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Dr.    0.3.   Strong 


DISEASES    OF    THE 
NERVOUS   SYSTEM 


Digitized  by  the  Internet  Archive 

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Diseases  of  the 
Nervous  System 


BY 

H.   CAMPBELL    THOMSON 
M.D.  (Lond.),  F.R.C.P. 

Physician  to  the  Department  for  Diseases  of  the  Nervous 
System,  and  Lecturer  on  Neurology,  Middlesex  Hospital; 
Physician  to  the  Hospital  for  Epilepsy  and  Paralysis,  Maida 
Vale;  formerly  Dean  of  the  Middlesex  Hospital  Medical  School 


Third  Edition,  Re-vised 


WITH   11   COLOUR  AND   12   BLACK-AND-WHITE 
PLATES  AND  120  FIGURES  IN  THE  TEXT 


New  York 
PAUL    B.    HOEBER 

67-69  East  59th  Street 
1921 


ALL    RIGHTS    RESERVED 


TO 

SIR  JAMES    KINGSTON   FOWLER,  K.C.V.O.,  C.M.G, 

IN   GRATEFUL  APPRECIATION   OF 
HIS     TEACHING     AND     COUNSEL 


PREFACE  TO  THE  THIRD    EDITION 

Ix  preparing  this  manual  for  a  Third  Edition,  I  have 
endeavoured  to  incorporate  such  new  matter  as  is  essen- 
tial without  materially  increasing  the  size  of  the  book. 

The  knowledge  obtained  by  the  researches  of  Henry 
Head,  Gordon  Holmes,  Sir  W.  Lister,  Riddoch,  and 
others,  during  the  War,  especially  in  connection  with 
the  effects  of  injuries  to  the  spinal  cord,  the  theory  of 
aphasia,  and  the  functions  of  the  cerebral  cortex  regard- 
ingf  sensation  and  vision,  has  necessitated  the  addition 
of  not  a  little  that  is  new  and  the  modification  of 
much  that  is  old. 

Among  other  subjects  now  introduced  into  the  book 
is  Encephalitis  Lethargica.  The  relation  between  the 
Neuroses  and  the  internal  secretions  has  been  empha- 
sized, and  a  fuller  description  given  of  the  Autonomic 
System. 

The  literature  of  the  Psychoneuroses  has  become  so 

extensive  that  it  is  difficult  to  deal  with  the  subject  in 

the  space  at  my  disposal,  and  in  largely  rewriting  and 

rearranging  this  section  I  have  contented  myself  with  an 
*  vii 


viii  PREFACE  TO  THE  THIRD  EDITION 
endeavour  to  present  the  more  salient  points  in  such 
perspectiv^e  as  will  enable  the  student  to  pursue  his 
investigations  in  any  branches  which  he  may  desire  to 
explore  further.  Arguments  as  to  the  relative  values 
of  the  different  schools  of  psychotherapy  I  have  thought 
it  well  to  avoid. 

I  am  greatly  indebted  to  Prof.  James  Mcintosh, 
Dr.  George  Riddoch,  Dr.  Percy  Whittick,  and  to  many 
other  friends  and  colleagues,  for  the  advice  and  assist- 
ance which  they  have  kindly  given  me. 

H.  C.  T. 

31,  Queen  Anne  Street,   \V.1. 
June,  1921. 


PREFACE  TO  THE  FIRST  EDITION 

In  attempting  to  marslial  the  main  facts  of  neurology 
in  a  concise  and  yet,  I  hope,  readable  manner,  it  has 
been  my  constant  aim  to  bear  in  mind  the  principal 
difficulties  which  are  apt  to  beset  the  student. 

The  experience  on  which  this  book  is  founded  has 
been  obtained  for  the  most  part  at  the  Hospital  for 
Nervous  Diseases,  Maida  Vale,  and  at  the  clinics  for 
nervous  diseases  which  I  have  for  some  years  past 
held  at  the  Middlesex  Hospital.  To  my  colleagues  at 
both  these  hospitals  I  express  my  hearty  thanks  for 
the  many  opportunities  they  have  kindly  given  me  of 
observing  cases  under  their  care. 

To  the  courtesy  and  generosity  of  Professor  Pierre 
Marie  in  supplying  me  with  photographs  for  illustra- 
tions I  owe  a  special  debt  of  gratitude,  as  also  I  do  to 
Mr.  Bland-Sutton  for  the  loan  of  blocks,  and  to  others 
who  have  kindly  allowed  me  to  reproduce  illustra- 
tions, the  sources  of  all  of  which,  I  trust,  are  duly 
acknowledged. 

For  taking  photographs  of  my  cases  I  have  chiefly 

ix 


X  PREFACE   TO   THE   FIRST   EDITION 

to  thank  Mr.  Mann,  and  for  the  taking  of  the  instan- 
taneous photographs  reproduced  in  four  of  the  plates 
I  am  indebted  to  Mr.  Charles  Urban. 

Finally,  my  best  thanks  are  due  to  the  publishers 
for  the  valuable  assistance  they  have  given  me  through- 
out the  preparation  of  the  book. 

H.  Campbell  Thomson. 

April,  1908. 


CONTENTS 

Section  I. — Introductory 

CHAPTER  PAGE 

1.  General  Structure  of  the  Nervous  System        1 

2.  General  Classification  of  Neurones        .         5 

3.  The  Eeflexes    .         .         .         .         .         .25 

4.  elgidity  and  contractures       ...      39 

5.  Electrical  Ee actions        ....       43 

6.  The  Autonomic  System  (Sympathetic  and 

Parasympathetic  Systems)      ...       46 

7.  General  Functions  of  the  Brain     .         .       54 

8.  Examination  of  the  Higher  Functions  of 

THE  Nervous  System      ....       66 

9.  Paths  of  Infection  of  the  Central  Nerv- 

ous System     ......       72 

10.  Lumbar  Puncture 76 

Section  II. — Diseases  of  the  Peripheral 

Nerves 

11.  Paralysis  of  Cranial  Nerves    ...       79 

12.  Paralyses  of  the  Upper  Limb  .         .         .115 


xii  CONTENTS 


PAGE 


13.  Paralyses  of  the  Lower  Limb  .         .     140 

14.  Multiple  Neuritis     .....     150 

15.  Tumours  of  Nerves  (Neuromata)      .         .162 

Section  III. — Muscular  Dystrophies 

16.  The  Myopathies  ;    Amyotonia  Congenita  ; 

Peroneal  Type  of  Atrophy  .         ,         .165 

Section  IV. — Diseases  of  the  Spinal  Cord 

17.  Classification .179 

18.  Paraplegia  and  Bulbar  Paralyses  .         .     183 

19.  Acute  Poliomyelitis  (Infantile  Paralysis)     189 

20.  Landry's  Paralysis 198 

21.  Myelitis 201 

22.  Subacute  Combined  Degeneration    .         .     207 
i3.  Disseminated  Sclerosis      .         .         .         .210 

24.  Progressive  Muscular  Atrophy  and  Pro- 

gressive Bulbar  Paralysis    .         .         .219 

25.  Spastic  Paraplegia  ;  Amyotrophic  Lateral 

Sclerosis  ;  Ataxic  Paraplegia        .         .228 

26.  Friedreich's  Disease  (Hereditary  Ataxy) 

AND  Hereditary  Spastic  Paraplegia      .     239 

27.  Syringomyelia 246 

28.  Spina  Bifida  ;    H^matomyelia  .  .         .     251 


CONTENTS  xiii 

CHAPTER  PAGE 

29.  Tumours  of  the  Spinal  Cord    .         .         .     254 

30.  Spinal  Meningitis  and  Meningeal  Hemor- 

rhage    .         .         .         .         .         .         .     261 

Section  V. — Syphilis  of  the  Nervous  System 

31.  Interstitial  and  Parenchymatous  Syphilis    266 

32.  General   Paralysis    op   the   Insane    (De- 

mentia Paralytica)         .         .         .         .278 

33.  Tabes  Dorsalis  (Locomotor  Ataxy)  .         .     284 

Section  VI. — Diseases  of   the  Brain,  and 
Diseases  of  General  Origin 

34.  Meningitis 312 

35.  Cerebral  Palsies  of  Children  ;  Amaurotic 

Family  Idiocy  ;    Hydrocephalus    .         .  329 

36.  Acute  Encephalitis  ;    Cerebral  Abscess  .  341 

37.  Intracranial  Tumours       .         .         .         .351 

38.  Diseases  of  the  Pituitary  Body       .         .  375 

39.  Progressive  Lenticular  Degeneration     .  386 

40.  Cerebral  Hemorrhage  ;   Cerebral  Throm- 

bosis ;    Cerebral  Embolism    .         .         .  387 

41.  Thrombosis  of  Cerebral  Sinuses       .         .  408 

42.  Aphasia 411 

43.  Myasthenia    Gravis    (Asthenic    Bulbar 

Paralysis) 421 


xiv  CONTENTS 

CHAPTKR  PAGE 

41.  Convulsions       ......  428 

45.  Epilepsy 432 

46.  Tetany 449 

47.  Paralysis  Agitans  (Parkinson's  Disease)  .  452 

48.  Chorea    (Rheumatic    Chorea,    Sydenham's 

Chorea,  St.  Vitus's  Dance)    .         .         .  460 

49.  The  Tics .467 

50.  Myoclonus  ;  Myotonia  Congenita  ;  Family 

Periodic  Paralysis         .         .         .         .471 

51.  Occupation  Neuroses         ....  475 

52.  Psychoneuroses         .....  479 

53.  Neuralgia  ;  Headache;  Migraine      .         .  513 

54.  Vertigo      .......  527 

55.  Vaso-motor  Neuroses        ....  531 

56.  Exophthalmic   Goitre   (Graves's    Disease, 

Basedow's  Disease)        ....  536 

57.  Tetanus  (Trismus,  Lockjaw)  ;  Hydrophobia  542 
INDEX 549 


LIST   OF   PLATES 

FACING   PAGE 

Plate  1  (Colour)       ......         5 

Motor    Field    with    its    Two    Principal 
Orders  of  Projection  Fibres. 

Plate  2  (Colour) .6 

Diagram  of  Principal  and  Subsidiary 
Descending  Projection  Fibres. 

Plate  3  (Colour) 11 

Sensory  Field  with  its  Principal  Orders 
OF  Projection  Fibres. 

Plate  4  (Colour)       ......       49 

Diagram  of  Arrangement  of  Vertebral 
Sympathetic  System. 

Plate  5  (Colour)     ......   83 

The  Fundus  Oouli. 

Plate  6  .         . 118 

Skiagram  of  a  Cervical  Eib  which  gave 
rise  to  Symptoms  of  Pressure  on  the 
Lower  Roots  of  the  Brachial  Plexus. 

Plate  7 168 

"  High-stepping  "  Gait  in  Pseudo-hyper- 
trophic  Paralysis. 

XV 


xvi  LIST   OF   PLATES 

lACING    PAGE 

Plate  8 170 

Case  op  Myopathy  showing  the  Charac- 
teristic Attitude  of  Lordosis. 

Plate  9  (Colour)       .         .         .         .         .         .180 

Tracts  of  the  Spinal  Cord. 

Plate  10 202 

Gait  in  Paraplegia  following  Myelitis. 

Plate  11 213 

Instantaneous  Photograph  showing  Tre- 
mors IN  Disseminated  Sclerosis. 

Plate  12 246 

Cavities  and  Gliomatosis   of  the  Spinal 
Cord  in  Syringomyelia. 

Plate  13  {Colour) 254 

Diffuse  Sarcoma  of  the  Brain  and  Cord. 

Plate  14  .......     278 

Spirochetes  in  the  Brain  in  a  Case  of 
General  Paralysis  of  the  Insane. 

Plate  15 324 

Case  of  Posterior  Basic  Meningitis  show- 
ing Extreme  Retraction  of  the  Head. 

Plate  16 .     332 

Bilateral  Athetosis  in  Cerebral  Diplegia. 

Plate  17  {Colour) 337 

The  Fundus  Oculi  in  Amaurotic  Family 
Idiocy. 


LIST    OF   PLATES  xvii 

FACING   PAGE 

Plate  18  {Colour) 355 

Diagram    of    the    Convolutions    of    the 
Cerebral  Cortex. 

Plate  19  {Colour) 366 

The  Cranial  Nerves  at  the  Base  of  the 
Brain. 

Plate  20  {Colour) 368 

Tumour  at  the  Angle  of  the  Pons  and 
Cerebellum. 

Plate  21 391 

Section  of  the  Brain  showing  the  Anatomy 
OF  the  Internal  Capsule. 

Plate  22 399 

Characteristic  Gait  in  Hemiplegia. 

Plate  23  .......     467 

Successive   Stages    op    Stumbling   Move- 
ments IN  A  Coordinated  Tic. 


DISEASES    OF    THE    NERVOUS 
SYSTEM 

SECTION  I.-INTRODUGTORY 

CHAPTER   I 

GENERAL    STRUCTURE   OF    THE    NERVOUS 
SYSTEM 

The  nervous  system  is  composed  of  nerve  cells  sur- 
rounded and  supported  by  connective  tissue.  A  nerve 
cell  consists  of  a  cell  body  and  its  processes.  The 
processes  which  conduct  impulses  away  from  the  cell 
body  are  called  axons,  while  those  which  transmit 
impulses  towards  the  cell  body  are  known  as  dendrons, 
and  the  cell  body  together  with  all  its  branches  con- 
stitutes the  neui'one  of  Waldeyer.  The  neurone  is 
therefore  a  cell  which  has  become  specially  differentiated 
to  perform  the  function  of  conduction      (Fig.  1.) 

The  cell  body,  as  studied  by  Nissl's  method  of 
staining  with  aniline  dyes,  is  principally  made  up  of 
two  substances  which  are  qualified  as  chromatic  and 
achromatic  according  to  their  staining  properties. 

The  chromatic  substance  is  arranged  in  cube-shaped 
patterns  known  as  Nissl  bodies,  which  are  distributed 
in  a  fairly  regular  manner  throughout  the  cell  body. 
Although  this  tessellated  appearance  is  probably  largely 
due  to  the  hardening  reagents  used  in  the  preparation 
of  the  specimens,  since  it  does  not  appear  in  cells  that 
are  stained  during  life,  it  is  nevertheless  an  important 
guide  for  comparative  purposes  in  estimating  the  changes 
that  have  taken  place  in  the  cell  bodies  as  the  result  of 
disease.     There  is  some  uncertainty  as  to  the  function 

B 


2       DISEASES    OF   THE   NERVOUS   SYSTEM 


this  cliromatopliile  substance  fulfils,  but  it  is  generally 
tliouglit  to  be  connected  with  the  nutrition  and  energy 

of  the  cell.  It  undergoes 
various  changes  as  the  re- 
sult of  disease,  among  the 
more  common  being  loss 
of  substance  (chromatolysis) 
or  a  uniform  staining  in 
which  all  definition  of  the 
chromatophile  granules  is 
obscured. 

The  unstainable  or  achro- 
matic substance  consists  of  a 
delicate  network  of  fibres 
which  appear  to  connect  the 
axons  with  the  dendrons, 
and  is,  therefore,  presum- 
ably closely  connected  witli 
the  conduction  of  nerve  im- 
pulses. 

There  is  a  nucleus  gener- 
ally situated  near  the  middle 
of  the  cell  body,  and  in- 
side that  a  nucleolus  which 
usually  shows  up  well  by 
reason  of  its  darker  stain- 
ing. Pigment  also  may 
often  be  seen  in  the  cell 
body,  but  the  significance 
of  its  presence  is  uncertain. 
The  axon,  as  a  rule,  is 
single  and  of  greater  length 
than  the  dendron,  and,  as 
already  mentioned,  it  gener- 
ally conveys  impulses  away 
from  the  cell  body  and 
gives  off  branches,  known 
as  collaterals,  along  its  course.  Shortly  after  leaving  the 
cell  body  the  axon  becomes  encased  successively  with 


Fjg.    1.  —  Diagrammatic   repre- 
sentation of  a  neurone.   (Moral.) 

N,  nucleus  of  the  nerve  cell  ;  Fp, 
protoplasmic  prolongations  ;  i'«,  axon, 
or  axis  cylinder ;  Gvi,  sheath  of 
myelin  ;  Sch,  sheath  of  SchwaJin  ;  Kn, 
annulai-  node  ;  T,  terminal  ramilica- 
lions  of  the  axis  cylinder. 


THE   NEURONE    THEORY  3 

a  sheath  of  myeUa  and  a  neurilemma  or  sheath  of 
Schwann,  both  of  which  it  discards  a  little  before  it 
breaks  up  into  its  terminal  branches. 

Such  is  a  typical  neurone,  but,  of  course,  the  size  and 
shape  of  the  cell  bodies  and  the  length  and  thicki^ss  of 
their  branches  differ  very  greatly,  and  there  are  some 
neurones  whose  axis  cylinders  do  not  leave  the  grey 
matter,  and  do  not  in  those  circumstances  acquire  a 
medullated  sheath. 

According  to  the  neurone  theory  every  neurone  is 
a  separate  anatomical  unit  capable  of  Hving  and  dying 
independently  of  every  other  neurone,  and  the  processes 
of  one  have  no  physiological  connection  with  those  of 
another,  i.e.  the  branches  are  contiguous  but  not  con- 
tinuous, just  as  the  branches  of  trees,  however  intimately 
entwined  with  those  of  others,  depend  for  their  vitality 
upon  the  integrity  of  their  own  stems  and  roots. 

It  thus  comes  about  that  the  nervous  system  is 
made  up  of  an  infinite  number  of  neurones,  bound  up  by 
connective  tissue  into  various  bundles  and  tracts  ac- 
cording to  the  difierent  kinds  of  impulses  they  conduct. 
There  are,  for  example,  special  bundles  for  motor  im- 
pulses —  the  motor  tracts — and  others  for  sensory 
impulses  —  the  sensory  tracts — and  so  on;  but  the 
ultimate  elements  making  up  all  these  bundles  are 
simple  neurones. 

Doubt  has  occasionally  been  cast  upon  the  correct- 
ness of  the  neurone  theory,  by  which  every  neurone  is 
supposed  to  have  a  complete  individuality.  Possibly  it 
may  not  hold  good  m  all  the  lower  forms  of  animal  life, 
but  in  the  higher  forms  and  in  man  the  distribution  of 
nerve  degeneration,  w^hich  is  mainly  limited  by  the 
termination  of  cell  branches  of  particular  tracts,  and  the 
embryological  evidence  of  the  independent  develoj^ment 
of  different  units,  are  both  strongly  in  favour  of  its  truth. 

The  mechanism  by  which  impulses  pass  from  one 
neurone  to  another  is  not  accurately  known.  It  has 
been  thought  that  the  terminal  filaments  of  the  neurones, 
upon  which  little  buds  or  excrescences  can  often  be  seen, 


4       DISEASES   OF   THE   NERVOUS   SYSTEM 

are  capable  of  retraction  and  protrusion  in  an  amoeboid 
manner,  thereby  linking  up  or  breaking  connections,  as 
the  case  may  be ;  but  this  is  at  present  largely  a  matter 
of  conjecture. 

A  clear  conception  of  the  constitution  of  the  nervous 
system  as  made  up  of  collections  of  neurones  is  an  im- 
portant aid  to  the  understanding  of  all  nervous  diseases, 
for  it  enables  us  to  foretell  to  a  large  extent  the  course 
and  Hmits  of  degenerations  and  to  understand  how 
certain  tracts  may  be  affected  to  the  exclusion  of 
others. 


Pyr.teU 


Ginie.  ir. 


Turek't  eotumn 
(ant.  pi/T.i 


Radicular  cM 


iluicui  titnttU 


A.luJ"^ 


PLATE   1.— Motor  field  with  its  two  principal  orders  of 
projection  ftbi'es.    {Moral.) 


CHAPTER    II 

GENERAL    CLASSIFICATION    OF    NEURONES 

The    neurones    may    be    divided    into    the    following 
groups,  viz.  : — 

1.  Projection  neurones. 

2.  Association  neurones. 

3.  Commissural  neurones. 

The  projection  neurones  connect  the  brain  with  the 
spinal  cord,  and  are  divided  again  into  descending  and 
ascending  groups  according  as  they  conduct  impulses 
from  the  brain  downwards  or  from  the  cord  upwards. 
Examples  of  the  down-going  neurones  are  to  be  found  in 
the  crossed  pyramidal  tracts,  the  cell  bodies  of  which 
are  situated  in  the  cerebral  cortex,  and  of  the  up-going 
neurones  in  the  long  fibres  of  the  posterior  columns  and 
in  those  of  the  cerebellar  tracts.     (Plates  1  and  3.) 

The  association  neurones  link  one  portion  of  the 
cortex  of  the  brain  with  another,  and  in  the  spinal  cord 
also  there  are  fibres  of  different  lengths  which  join  up 
various  segments.     {See  Fig.  8,  p.  55.) 

The  commissural  neurones  connect  the  two 
segments  of  the  brain  and  the  two  halves  of  the  cord 
with  each  other,  e.g.  the  fibres  of  the  corpus  callosum 
which  bridge  the  space  between  the  two  hemispheres  of 
the  brain,  and  the  fibres  forming  the  commissure  which 
unites  the  two  halves  of  the  spinal  cord. 

DESCENDING   PROJECTION   FIBRES 
The  principal  descending  projection  fibres,  or  upper 
motor  neurones  as  they  are  called,  are  those  which 
make  up  the  motor  tract  down  which  pass  the  impulses 

5 


6       DISEASES   OF  THE  NERVOUS   SYSTEM 

that  give  rise  to  volimtiiiy  aiovements.  The  cell  bodies 
of  these  upper  motor  neurones  are  situated  in  the  jnotor 
area  of  the  cerebral  cortex,  and  while  their  short  branches 
(dendrons)  interlace  with  others  in  all  directions,  their 
long  ones  (axons)  converge  to  form  the  main  motor 
tract.  After  coming  of!  from  their  cell  bodies  in  the 
cerebral  cortex  these  fibres  pass  successively  through 
the  corona  radiata,  internal  capsule,  crus  cerebri,  pons, 
and  medulla.  At  the  level  of  the  crus  and  the  pons  some 
of  the  fibres  leave  the  main  body,  and,  after  decussating 
w4th  their  fellows  of  the  opposite  side,  terminate  round 
the  motor  cells  of  various  cranial  nerves.  At  the  medulla 
nearly  all  the  remainder  of  the  fibres  decussate  with 
their  fellows  of  the  opposite  side  and  then  pass  down  the 
cord  as  the  crossed  pyramidal  tracts,  where  they  terminate 
at  different  levels  by  coming  into  relationship  with  the 
branches  of  the  large  motor  cells  of  the  anterior  cornua. 
This  communication  with  the  cells  of  the  cord  is  not  a 
direct  one,  but  appears  to  be  established  through  short 
intermediary  neurones  in  the  grey  substance  of  the  cord. 

Those  fibres  which  do  not  decussate  at  the  medulla 
travel  down  the  anterior  columns  of  the  cord  on  the 
same  side  and  form  the  direct  pyramidal  tracts.  Their 
destination  is  likewise  the  cells  of  the  anterior  cornua, 
and  as  they  descend  the  cord  they  also  cross  to  the 
cells  of  the  opposite  horns.  These  fibres  have  generally 
all  reached  their  destinations  by  the  time  they  have 
arrived  at  the  midthoracic  region  of  the  cord,  below  the 
level  of  which  the  direct  pyramidal  tract  cannot  usually 
be  traced. 

The  motor  paths  are  continued  from  the  anterior 
horns  outwards  to  the  muscles  by  a  secondary  set  of 
projection  fibres  known  as  the  lower  motor  neurones, 
whose  cell  bodies  are  situated  in  the  grey  matter  of 
the  anterior  horns.  The  course  of  these  two  sets  of 
descending  projection  fibres  is  depicted  in  the  accom- 
panying plate  (Plate  1). 

In  addition  to  the  main  tracts  just  described  there 
arc  some  subsidiary  tracts  which  also  conduct  impulses 


SE^UCmanAR   t>VN>VL5. 


PLATE  2.— Diagram  of  principal  and  subsidiary  descending  projection 

fibres. 


SUBSIDIAHY   MOTOR   TRACTS  7 

downwards,   and  wlik'li   may  possibly  serve  in  certain 
circumstances   as   additional   paths   for  the   passage   of 
voluntary  motor  impulses. 
The  chief  of  these  are — 

1.  The  rubro-spinal  tract  (Monakow's  bundle). 

2.  The  thalamo-spinal  tract. 

3.  The  tecto-spinal  tract. 

4.  The  vestibulo-spinal  tract. 

1.  The  fibres  of  the  rubro-spinal  tract  arise  from 
the  red  nuclei  which  are  situated  in  the  tegmentum  of 
the  crura  cerebri.  They  immediately  decussate  with 
their  fellows  of  the  opposite  side,  and  then  each  tract 
passes  dow^n  the  lateral  columns  of  the  cord  in  close 
proximity  to  and  just  in  front  of  the  crossed  pyramidal 
tract.  The  rubro-spinal  tract  also  receives  impulses  from 
the  cerebellum  by  fibres  which  pass  by  way  of  the 
superior  cerebellar  peduncle  to  the  red  nucleus. 

2.  The  thalamo-spinal  tract  is  composed  of  fibres 
which  arise  from  the  thalamus  and  accompany  the  fibres 
of  the  rubro-spinal  tract  downwards  into  the  cord. 
Fibres  from  the  lenticular  nucleus  also  probably  accom- 
pany this  tract. 

3.  The  tecto-spinal  tract  takes  its  origin  from  the 
region  of  the  midbrain,  and  after  decussation  with  the 
fibres  from  the  opposite  side  passes  down  the  anterior 
columns  of  the  spinal  cord,  where  it  probably  terminates 
at  different  levels  in  relation  with  the  cells  of  the  anterior 
cornua. 

4.  The  fibres  of  the  vestibulo-spinal  tract  arise 
from  Deiters'  nucleus,  which  is  situated  in  the  pons. 
They  do  not  decussate,  but  each  bundle  passes  down  the 
anterior  column  of  the  cord  on  its  own  side  and  terminates 
in  connection  with  the  cells  of  the  anterior  horns.  Owing 
to  the  connection  which  Deiters'  nucleus  has  with  the 
vestibular  nerve  through  nuclei  of  the  cerebellum,  the 
vestibulo-spinal  tract  probably  serves  an  important 
function  in  conducting  impulses  which  assist  in  keeping 
up  the  tone  of  the  muscles.  It  is  thus  seen  that  the 
cerebellum   is   able   to   exercise   a   direct   influence    on 


8       DISEASES   OF   THE  NERVOUS   SYSTEM 

the  functions  of  the  cord  tlirougli  (1)  the  rubro-spinal 
tracts  by  way  of  the  superior  cerebellar  peduncles  and 
(2)  the  vestibulo-spinal  tracts  by  their  communications 
with  Deiters'  nuclei. 

CLINICAL  IMPORTANCE  OF  THE  DESCENDING 
OR   MOTOR   TRACTS 

It  has  already  been  stated  that  nearly  all  the  im- 
pulses which  give  rise  to  voluntary  movements  travel 
along  the  main  descending  projection  tract  known  as 
the  motor  tract,  the  fibres  of  which  pass  from  the  cell 
bodies  in  the  motor  area  of  the  cerebral  cortex,  through 
the  corona  radiata,  internal  capsule,  crus  cerebri,  pons, 
and  medulla  (where  they  decussate),  and  thence  down  the 
crossed  pyramidal  tracts  of  the  cord  to  reach  the  cells 
of  the  anterior  horn.  Here  the  fibres  terminate,  and  the 
second  relay,  or  lower  motor  neurone  as  it  is  called, 
begins  and  forms  the  rest  of  the  path  for  conducting  the 
motor  impulses  out  to  the  muscles. 

The  tract  is  thus  divided  into  two  segments  consist- 
ing of  (1)  the  upper  motor  neurone,  which  begins  in 
the  cerebral  cortex  and  ends  in  relation  with  the  cells  of 
the  anterior  horn,  and  (2)  the  lower  motor  neurone, 
which  has  its  beginning  in  the  cells  of  the  anterior  horn 
and  its  ending  in  the  muscles.  Since  almost  all  im- 
pulses which  give  rise  to  voluntary  movements  pass 
down  these  tracts,  it  follows  that  any  break  in  the  course 
of  the  fibres  of  either  of  these  neurones  will  be  accom- 
panied by  some  loss  of  voluntary  power,  or,  to  put  the 
matter  in  its  clinical  aspect,  a  patient  with  loss  of 
voluntary  power  must,  except  in  the  case  of  functional 
disorders,  have  a  lesion  somewhere  in  the  course  of  the 
motor  tracts,  or  in  the  muscles  themselves,  which  pre- 
vents the  impulses  from  passing. 

Once,  therefore,  the  fact  of  loss  of  power  is  established, 
the  next  step  is  to  endeavour  to  decide  whether  the 
interruption  lies  in  the  course  of  the  upper  or  of  the 
lower  neurone  of  the  tract,  and  for  this  purpose  certain 
diagnostic  differences  must  be  borne  in  mind. 


UPPER   AND   LOWER   MOTOR   NEURONES     9 

The  impulses  whicli  travel  down  the  upper  neurone, 
besides  giving  rise  to  voluntary  movements,  also  exercise 
restraint  over  the  actions  of  the  lower  neurone,  so  that 
when  this  restraint  is  removed  there  is  found,  in  addition 
to  loss  of  power,  some  rigidity  of  muscles  and  increase 
of  the  deep  reflexes  in  the  affected  parts.  But,  inasmuch 
as  the  lower  neurone  is  independent  of  the  upper  as  far 
as  its  actual  existence  is  concerned,  there  is  no  wasting 
of  muscles  except  such  as  occurs  from  disuse,  neither 
is  there  any  change  in  their  electrical  reactions.  On 
the  other  hand,  if  the  lower  neurone  is  injured  there  is 
not  only  loss  of  power  but  also  loss  of  reflexes  (if  any 
happen  to  be  situated  in  the  affected  zone),  together 
with  wasting,  flaccidity,  and  changes  in  the  electrical 
reactions  of  the  muscles,  for  the  nutrition  of  the 
muscles  is  directly  dependent  on  the  vitality  of  the 
lower  segment. 

These  differences  may  be  conveniently  summarized 
in  the  following  table  : — 


1.  Wasting    .. 

2.  Reflexes    .. 

3.  Rigidity    .. 

4.  Electrical 

reactions 


Lesion  of  Upper 
Neurone 


Slight,  and  only  in  con- 
sequence of  disuse 

Deep  reflexes  increased ; 
plantar  reflex  gives  an 
"extensor  response"; 
superficial  reflexes  of 
abdomen  often  lost 

Limbs  tend  to  become 
rigid 

No  obvious  changes 


5,  Cow^raciwres  Rigidity-accompanied  by 
contractures  in  which 
the  arm  tends  to 
become  flexed  and 
adducted  and  the  leg 
usually  extended 


Lesion  of  Lower 
Nedrone 


Wasting     a     prominent 

feature. 
Abolished  in  the  affected 

segments. 


Limbs  tend  to  become 
flaccid. 

Reactions  to  faradism 
and  galvanism  modi- 
fied. Frequently  a 
typical  "  reaction  of 
degeneration." 

Irregular  deformities 
owing  largely  to  unop- 
posed actions  of  non- 
paralysed  muscles. 


10     DISEASES   OF   THE   NERVOTJS   SYSTEM 

It  will  ])e  seen  that  increased  reflexes  and  rigidity 
tend  to  go  together,  while  flaccidity,  wasting,  and  loss  of 
reflexes  generally  accompany  one  another. 

The  importance  of  this  table  to  the  student  is  that 
in  it  he  has  the  basis  of  all  the  symptoms  which  depend 
upon  loss  of  power,  and  instead  of  endeavouring  to 
remember  a  different  set  of  points  for  every  individual 
instance  he  can  apply  the  general  principles  to  his  case 
and  then  vary  the  particulars  as  required. 

For  instance,  the  motor  symptoms  underlying  primary 
or  secondary  lateral  sclerosis  and  ataxic  paraplegia  wall 
all,  in  so  far  as  they  depend  upon  a  lesion  of  the  upper 
neurone,  obey  the  general  rules  of  such  lesions.  Modifi- 
cations wall  merely  concern  methods  of  onset,  course, 
and  similar  details.  On  the  other  hand,  poliomyelitis, 
progressive  muscular  atrophy,  and  peripheral  neuritis 
have  their  motor  symptoms  founded  on  the  principles 
of  a  lower  neurone  lesion. 

Sometimes  the  two  conditiojis  are  mixed,  as  in 
amyotrophic  lateral  sclerosis,  where  the  usual  clinical  pic- 
ture is  that  depending  upon  a  lesion  of  the  lower  neurone 
as  far  as  the  hands  are  concerned,  and  upon  one  of  the 
upper  neurone  as  regards  the  legs,  which  signifies  that 
the  cells  of  the  anterior  horns  in  the  cervical  region 
of  the  cord  are  degenerating  at  the  same  time  as  those 
fibres  of  the  crossed  pyramidal  tracts  w^hich  carry  volun- 
tary impulses  to  the  legs. 

The  symptoms  of  disease  of  the  subsidiary  dc- 
scendincj  tracts  are  less  clear,  but  lesions  wdiich  in- 
clude the  rubro-spinal,  thalamo-spinal,  and  tecto-spinal 
fibres  appear  to  be  followed  by  some  generalized  mus- 
cular weakness,  rigidity  and  tremors.  If  the  lesion  is 
above  the  red  nucleus  so  that  the  thalamo-spinal  and 
tecto-spinal  fibres  suffer  while  the  rubro-spinal  tract 
escapes,  the  principal  result  seems  to  be  a  tendency 
to  develop  tremors.  On  the  other  hand,  when  the 
Vestibulo-spinal  and  other  descending  cerebellar  tracts 
are  affected,  the  principal  results  are  loss  of  muscle  tone 
and  incoordination  of  movement.     Perfection  of  volun- 


PiOet 

Ruban  de  ReU ' 


Cran.  szntory  n. 
~Sens.  deoiuiation. 


N.  of  Ooll  and  of 
Burdach. 


Column  of  OoU. 
Anterior  eoUmmn. 


■Lateral  column. 


Ant.  eommiti. 


Pott.  corn. 


Spinal  v 


J-S-u^i' 


PLATE  8.— Sensory  field  with  its  principal  orders  of  projection 

fibres.     (.Moral.) 


SENSORY  NEURONES  11 

tary  movement  depends,  therefore,  upon  the  integrity 
of  all  the  descending  tracts,  and  if  any  one  of  these  is 
destroyed  the  balance  is  upset,  with  a  resulting  disorder 
of  movement  due  partly  to  loss  of  function  of  the 
diseased  tract  and  partly  to  unantagonized  over-action 
of  the  tracts  that  remain.  The  symptoms  of  such  a 
lesion  are  thus  divisible  into  two  groups — (1)  negative 
sym2Dtoms  due  to  loss  of  function  of  the  diseased  parts, 
(2)  positive  symptoms  due  to  removal  of  control  and 
consequent  over-action  of  other  systems. 

For  example,  in  a  case  of  hemiplegia  due  to  a  lesion 
of  the  internal  capsule  the  negative  effects  are  shown 
by  the  inability  to  perform  certain  movements  volun- 
tarily— i.e.  by  '"' jDaralysis  " — while  the  positive  results 
are  seen  in  the  rigidity,  increased  tendon  reflexes,  and 
other  symptoms  due  to  unantagonized  action  of  the 
cerebellar  system  ;  in  some  instances  further  positive 
results  occur  in  the  shape  of  involuntary  movements 
(athetosis)  due  to  unrestrained  activity  of  the  midbrain 
subsidiary  motor  systems.  These  instances  illustrate 
an  im23ortant  principle,  first  enunciated  by  Hughlings 
Jackson,  that  a  destructive  lesion  in  any  part  of  the 
nervous  system  tends  to  produce  negative  results  due  to 
impaired  function  of  the  part  affected,  and  positive 
results  due  to  loss  of  control  of  the  injured  part  over 
other  parts  which  are  lower  in  the  functional  scale. 

SENSORY    NEURONES 

The  neurones  for  the  conduction  of  sensations  are  more 
numerous  and  arranged  in  a  more  complicated  manner 
than  are  those  for  movements.  It  is  necessary  to  con- 
sider their  arrangement  and  the  effects  produced  by 
their  injury  or  disease  (1)  in  the  peripheral  nerves,  (2)  in 
the  posterior  roots,  (3)  in  the  spinal  cord,  and  (4)  in  the 
brain. 

1.  Sensory  Neurones  in  the  Peripheral  Nerves 
Head,  Rivers,  and  Sherren,  and  Head  and  Sherren, 
have  shown  that  in  the  peripheral  system  the  afferent 


12     DISEASES   OF   THE   NERVOUS   SYSTEM 

nerves  can  be  divided  into  three  systems — (i.)  the  system 
for  deep  sensibility,  (ii.)  the  protopathic  system,  and  (iii.) 
the  epicritic  system. 

i.  In  the  system  for  deep  sensibility,  sensations 
arising  from  pressure  and  from  movements  of  tendons, 
joints,  and  muscles  are  conducted. 

The  locality  of  the  stimulus  and  the  direction  of 
movement  in  a  joint  can  be  appreciated,  as  also  can 
pain  if  the  pressure  is  excessive  or  if  a  joint  is  injured. 
The  fibres  which  conduct  the  impulses  for  this  form 
of  sensibility  run  chiefly  with  the  nerves  to  the  muscles, 
and  they  are  not  destroyed  by  dividing  all  the  sensory 
nerves  to  the  skin. 

Thus,  when  the  sensation  to  light  touch  is  entirely 
lost  in  the  skin,  a  sense  of  'pressure  and  of  inovements 
of  joints  may  still  be  appreciated  provided  the  deep  system 
is  intact. 

ii.  In  the  protopatliie  system  the  fibres  respond 
to  painful  cutaneous  impressions  and  to  the  extremes 
of  heat  and  cold. 

iii.  In  the  epicritic  system  accurate  cutaneous 
localization  of  light  touch,  the  discrimination  of  the 
points  of  a  compass,  and  the  recognition  of  the  slighter 
differences  in  temperature  are  accomplished. 

A  knowledge  of  the  existence  of  these  three  systems 
is  obviously  of  the  greatest  importance  in  practical 
medicine. 

In  th(i  first  place  it  shows  what  care  it  is  necessary 
to  take  in  examining  for  sensibility,  for  when  cutaneous 
nerves  are  severed  an  appreciation  of  slight  pressure 
through  the  deep  system  may  easily  lead,  as  it  often 
has  done  in  the  past,  to  an  erroneous  supposition  that 
there  is  no  anaesthesia.  In  testing  for  the  tactile 
sensibility  of  the  skin  the  very  lightest  form  of  stimu- 
lation, such  as  can  be  obtained  with  cotton-wool,  is 
required. 

Moreover,  the  loss  of  protopathic  and  of  epicritic 
sensibility  is  not  always  equal  in  extent  or  intensity. 
When,  for  instance,  union  is  taking  place  between  the 


SENSORY   NEURONES  13 

ends  of  a  divided  nerve  the  sensation  to  painful  stimuli 
returns  first.  For  a  time  the  pain  cannot  be  localized, 
but  radiates  widely  from  the  spot  stimulated,  though 
always  in  definite  directions. 

Together  with  return  of  sensibility  to  pain  there  is 
also  a  return  of  appreciation  of  extremes  of  temperature, 
e.g.  great  heat  or  great  cold,  which  Head  has  shown  to 
be  due  to  the  presence  of  "  heat  "  and  "  cold  "  spots. 
With  the  return  of  sensation  for  pain  the  nutrition  of 
the  sldn  also  improves,  and  any  ulcers  that  may  have 
been  present  readily  heal.  Gradually  the  skin  over  the 
afiected  areas  again  becomes  sensitive  to  light  touch 
and  to  slight  differences  of  temperature. 

It  therefore  appears  that  the  appreciation  of  touch 
and  of  pain  is  subserved  by  independent  fibres,  since 
Head  has  been  able  to  demonstrate  the  dissociation  of 
these  two  forms  of  sensation,  and  also  that  fibres  which 
conduct  the  painful  impressions  are  capable  of  regener- 
ating more  quickly  than  those  which  conduct  the  tactile 
impulses.  Head  has  summarized  the  characteristics  of 
the  three  systems  as  follows  :* — 

Loss  of  epicritic  sensibility  abolishes — 

Recognition  of  light  touch  over  hairless  parts  or 

parts  that  have  been  shaved. 
Cutaneous  localization. 
Discrimination  of  compass  points. 
Appreciation  of  differences  of  size,  including  the 

accurate  discrimination  of  the  head  from  the 

point  of  a  pin  apart  from  the  pain  of  the  prick 

(acusesthesia); 
Discrimination     of      intermediate      degrees      of 

temperature    from    about    25°     C.    to     about 

40°  C. 
Loss  of  protopathic  sensibility  abolishes — 

Cutaneous    pain,    especially    that    produced    by 

pricking,    burning,    or   freezing,   together   with 

that  of  stimulation  with  a  painful  interrupted 
*  £rain,  Part  cxvi.,  1906. 


14     DISEASES   OF   THE   NERVOUS   SYSTEM 

current.     Over   hair-clad    parts,    plucking   the 
hairs  ceases  to  be  painful. 
Sensations  of  heat  from  temperatures  higher  than 

about  45°  C. 
Sensations  of  cold  from  temperatures  below  20°  C. 

After  destruction  of  all  cutaneous  afferent  fibres  the 
'part  is  still  endoived  with  deep  sensibility. 

Pressure  can  be  recognized,  and  its  gradual  increase 
appreciated.  Pain  is  produced  by  excessive  pressure 
(measured  by  the  algometer).  Movements  of  muscles 
can  be  recognized. 

The  point  of  application  of  pressure  can  be  localized. 

The  patient  can  recognize  the  extent  and  direction  of 
movement  produced  passively  in  all  the  joints  within 
the  affected  atea. 

Muscle  sense. — Sensations  from  muscles,  joints, 
and  ligaments  are  generally  grouped  together  under 
the  term  "  muscle  sense,"  and  their  loss,  singly  or  com- 
bined, is  largely  responsible  for  the  incoordination  in 
locomotor  ataxy. 

The  sense  of  the  muscles  can  be  tested  by  finding 
whether  the  patient  can  distinguish  the  difiercnces  in 
weight  of  objects  of  similar  size;  and  allied  to  this  is 
the  powder  of  recognizing  with  accuracy,  by  contact,  the 
shapes  of  objects,  i.e.  the  stereognostic  sense. 

When  the  sensibility  of  the  joints  is  impaired  the 
patient  cannot  tell  in  what  position  his  limbs  are  placed, 
and  this  can  be  demonstrated  by  putting  the  limbs  in 
various  positions  and  requesting  him  (while  his  eyes 
are  closed)  to  describe  the  positions  or  to  put  his  other 
limbs  into  similar  positions. 

The  sensibility  of  the  bones  can  be  tested  by  means  of 
vibrations  produced  by  placing  on  them  a  tuning-fork. 
This  sensation,  like  those  from  the  muscles  and  joints, 
is  frequently  impaired  in  locomotor  ataxy. 

2.  Sensory  Neurones  in  the  Posterior  Roots 
The  distribution  of  anaesthesia  in  root  lesions  differs 
from  that  of  the  peripheral  nerves  inasmuch  as  the  fibres 


SENSORY  NEURONES   IN   THE   CORD      15 

are  so  arranged  that  each  root  corresponds  to  a  definite 
segment  of  skin.  The  segmentary  distribution  of  the 
posterior  roots  has  been  investigated  by  many  observers, 
and  with  exceptional  thoroughness  by  Head,  in  con- 
nection with  the  areas  occupied  by  the  eruption  of 
herpes  zoster,  an  affection  which  is  due  to  lesions  of  the 
posterior  root  ganglia.     (Figs.  2,  3.) 

Destination  of  the  fibres  of  the  posterior  roots. 
— The  principal  connections  which  the  fibres  of  the 
posterior  roots  make  after  entering  the  cord  may  be 
summarized  as  follows  : — 

i.  By  the  posterior  columns  of  the  cord  with  the 
nucleus  gracilis  and  nucleus  cuneatus  of  the  medulla, 
thence  by  the  mesial  fillet  to  the  optic  thalamus,  and 
thence  to  the  cerebrum  (this  is  the  path  for  con- 
scious muscle  sense  and  tactile  discrimination). 

ii.  With  the  cells  of  Clarke's  column,  and  thence  by 
dorsal  and  ventral  cerebellar  tracts  to  the  cerebellum 
(the  chief  paths  for  maintenance  of  equilibrium). 

iii.  With  the  cells  at  the  apex  of  the  posterior  horn, 
and  thence  by  the  spino -thalamic  tract  of  the  opposite 
side  to  the  thalamus  and  the  cerebrum  (the  path  for 
pain,  temperature,  and  diffuse  tactile  sensibility). 

iv.  With  the  cells  at  the  apex  of  the  posterior  horn  of 
each  side  in  the  same  and  distal  segments,  and  thence  to 
motor  cells  of  the  anterior  horn  (the  path  for  short  and 
long  spinal  reflexes). 

V.  With  the  cells  of  the  anterior  horn  direct  (probably 
the  path  for  tendon  reflexes). 

3.  Sensory  Neurones  in  the  Spinal  Cord 
The  principal  sensory  tracts  in  the  cord  are  the  pos- 
terior columns,  the  direct  cerebellar  tracts,  the  antero- 
lateral tracts  of  Gowers,  and  the  ascending  tracts  in  the 
anterior  columns. 

The  posterior  columns. — The  posterior  columns 
are  divided  into  posterior  external  (column  of  Burdach) 
and  posterior  internal  (column  of  Goll).  These  columns 
are  composed  mainly  of  fibres  which  are  a  continuation 


16     DISEASES   OF   THE   NERVOUS   SYSTEM 

of  those  forming  the  posterior  roots  (exogenous  fibres), 
and  to  a  small  extent  of  fibres  which  run  from  one  level 
of  the  cord  to  another  (endogenous  fibres). 


C6 


Fig.  2.— .Segmentary  bensory  areas  of  the  spinal  cord.  (After  Tooth.) 


THE   POSTERIOR   COLUMNS 


17 


Of  the  exogenous  fibres  (a)  a  large  number  pass 
directly  up  to  the  top  of  the  same  side  of  the  cord  and 
terminate  in  the  nucleus  gracilis  and  the  nucleus  cuneatus. 


Fig.  3.— Segmentary  sensory  areas  of  the  spinal  cord.    (After  Tooth. ) 
C 


18     DISEASES   OF   THE  NERVOUS   SYSTEM 

On  their  way  up  they  give  off  numerous  collaterals  which 
terminate  in  the  cells  of  the  grey  matter.  From  the 
nucleus  gracilis  and  nucleus  cuneatus  there  starts  a  second 
relay  of  fibres  which  cross  and  terminate  in  the  optic 
thalamus  of  the  opposite  side.  From  the  optic  thalamus 
springs  a  third  set  which  spreads  out  and  terminates  in 
the  cerebral  cortex.     (Plate  3.) 

The  chief  function  of  these  long  fibres  of  the  posterior 
columns  appears  to  be  that  of  conducting  impulses  for 
tactile  discrimination  (i.e.  compass  test)  and  for  the 
sensations  of  passive  movements  and  position  which 
rise  into  consciousness,  since  these  sensations  are  not 
lost  on  the  opposite  side  in  a  Brown-Sequard  lesion  of 
one  half  of  the  cord  (Head). 

Destruction  of  these  columns  is  usually  accom- 
panied by  some  ataxy,  though  the  influence  in  this 
direction  of  a  possibly  associated  lesion  of  fibres 
connected  with  the  cerebellar  tracts  must  not  be  lost 
sight  of. 

(b)  There  are  fibres  which  run  a  variable  distance 
up  the  cord  and  then  cross.  Their  function  seems 
mainly  to  be  that  of  conducting  tactile  impulses,  and, 
owing  to  the  distance  they  run  in  the  cord  before  crossing 
to  the  ascending  columns  of  the  opposite  side,  they  too 
arc  often  apt  to  escape  on  the  opposite  side  to  the  lesion 
in  a  Brown-Sequard  paralysis  (Head). 

There  are  also  fibres  which  pass  up  the  cord  some 
distance,  and  then  communicate  with  cells  at  the 
apex  of  the  posterior  horns  to  form  Lissauer's  tract. 

The  fibres  passing  direct  to  the  cells  of  the  an- 
terior horns  need  no  special  description. 

The  endogenous  or  propriospinal  fibres  of  the  posterior 
column  which  connect  different  segments  of  the  cord 
together  are  situated  chiefly  at  the  concavity  of  the  grey 
matter,  where  they  form  the  cornuo-commissural  bundle, 
and  near  the  se])tum  dividing  the  posterior  cclunms  of 
the  two  sides,  where  they  form  the  scpto-marginal  bundle. 

The  direct  cerebellar  tract. — The  fibres  of  the 
direct  cerebellar  tract  take  their  origin  from  cells  forming 


PATH   OF   IMPULSES   IN   THE   CORD       19 

Clarke's  column  wliich  are  situated  on  the  inner  side  of 
the  grey  matter  of  the  posterior  horn.  After  passing 
up  the  cord  the  fibres  reach  the  cerebellum  by  means  of 
the  inferior  cerebellar  peduncle  or  restiform  body,  and 
finally  end  in  the  vermis. 

The  origin  of  this  tract  is  mainly  confined  to  the 
dorsal  and  upper  lumbar  regions.  Its  lower  limit  has 
not  been  exactly  defined,  but  in  man  it  is  probably  about 
the  first  lumbar  segment,  and.it  does  not  increase  in 
volume  after  it  has  reached  the  first  dorsal  segment. 
The  fibres  of  this  tract  form  an  important  link  between 
those  of  the  posterior  roots  and  the  cerebellum. 

The  ascending  antero-lateral  tracts  com- 
prise various  sets  of  fibres;  the  principal  are — 

[a)  The  tract  of  Gowers,  or  ventral  cerebellar  tract, 
which  is  composed  of  fibres  arising  from  cells  in  the  grey 
matter  and  then  passing  upwards  by  way  of  the  superior 
peduncle  to  the  cerebellum,  where  they  terminate  in  the 
vermis.  The  function  of  these  fibres  is  similar  to  that 
of  the  fibres  of  the  direct  cerebellar  tract,  namely,  to 
conduct  impulses  from  the  deep  structures — bones, 
joints,  ligaments,  and  muscles — ^to  the  cerebellum.  Some 
fibres  in  this  tract  go  also  to  the  lateral  nucleus,  the 
corpora  quadrigemina,  and  the  optic  thalamus. 

(b)  A  tract  of  ascending  fibres  known  as  the  spino- 
thalamic tract,  and  situated  in  close  apposition  to  Gowers' 
tract.  Its  fibres  also  arise  from  cells  in  the  grey  matter, 
for  the  most  part,  of  the  opposite  side  of  the  cord,  the 
decussation  taking  place  in  the  anterior  commissure. 
The  destination  of  these  fibres  is  the  optic  thalamus, 
and  they  form  an  important  path  for  the  conduction  of 
crossed  tactile  impulses.  Temperature  and  pain  impulse 
also  reach  the  brain  by  fibres  of  the  antero-lateral  tracts. 

Path  of  impulses  in  the  cord. — ^It  has  been 
shown  by  Henry  Head  and  Theodore  Thompson*  that 
the  grouping  of  the  sensory  impulses  in  the  cord  is  quite 
different  from  that  of  the  peripheral  nerves,  in  which 
they  run  in  three  main  streams  by  the  epicritic,  proto- 
*  JBraiu,  Part  cxvi. 


20     DISEASES   OF   THE  NERVOUS   SYSTEM 

pathic,  and  deep  sensibility  fibres.  In  the  cord  the 
impulses  for  the  different  forms  of  sensation  tend  to 
unite,  irrespectively  of  the  various  paths  by  which  they 
have  arrived.  Thus  the  tactile,  painful,  and  thermal 
sensations  are  all  collected  together  in  their  own  groups, 
and  the  thermal  sensations  are  further  particularized 
into  those  for  heat  and  cold,  so  that  in  some  cases  the 
sensations  for  one  may  be  lost  while  those  for  the  other 
are  retained.  This  new  grouping  appears  to  take  place 
on  the  same  side  of  the  cord  as  that  by  which  the  impulses 
enter,  but  after  the  new  formation  has  taken  place  those 
of  tactile  sensibility,  tactile  localization,  and  painful  and 
thermal  sensibility,  all  cross  sooner  or  later  to  the  other 
side.  But  those  impulses  which  are  concerned  with  the 
knowledge  of  the  position  of  the  body,  spatial  discrimina- 
tion as  tested  with  the  points  of  a  compass,  apprecia- 
tion of  size,  form,  and  weight,  and  those  conveying  the 
sensation  of  vibration  as  produced  by  a  tuning-fork,  pass 
straight  up  the  long  fibres  of  the  posterior  columns  of 
the  same  side  to  the  gracilis  and  cuneate  nuclei.  They 
then  cross  at  the  sensory  decussation  and  pass  up- 
wards to  the  optic  thalamus,  which  part  of  their  course 
is  dealt  with  more  particularly  in  the  following  section. 

The  main  types  of  cord  anaesthesia  that  can  be 
recognized  are  those  arising — 
From  transverse  lesions. 
From  lesions  of  the  grey  matter. 
From  lesions  of  one  half  of  the  cord. 

In  lesions  which  extend  across  the  cord,  all  forms  of 
sensibility  will  be  affected  below  the  injury.  If  the 
lesion  is  complete,  there  will  be  total  anaesthesia  below  its 
level,  with  probably  a  zone  of  hypersesthesia  just  above, 
owing  to  irritation  and  inflammation  of  the  posterior 
roots  in  the  immediate  vicinity  of  the  injury. 

With  disease  of  the  commissural  grey  matter  there 
is  very  apt  to  be  loss  of  sensation  of  pain  and  tempera- 
ture, while  that  of  touch  is  preserved — a  dissociation 
particularly  well  seen  in  cases  of  syringomyelia. 

When  injury  or  disease  is  sharply  limited  to  one  half 


REFERRED   PAINS  21 

of  the  cord,  as  may  occur  in  stab  or  bullet  wounds,  the 
symptoms  known  as  Brown-Sequard  paralysis  are  liable 
to  follow  :  on  the  side  of  the  lesion  and  below  its  level, 
loss  of  power,  loss  of  sense  of  position,  of  tactile  dis- 
crimination* and  of  vaso-motor  tone,  while  on  the  oppo- 
site side  there  is  loss  of  sensation  to  touch  (partly),  and 
loss  of  pain  and  temperature  senses.  Head  has  shown 
that  while  the  loss  of  pain  and  temperature  senses  is 
constant  in  these  cases,  those  of  touch  and  pressure  are 
more  rarely  affected — due,  presumably,  to  their  crossing 
to  the  other  side  of  the  cord  less  rapidly  than  the  others. 

At  the  level  of  the  lesion,  and  on  the  same  side,  there 
is  a  zone  of  anaesthesia  corresponding  to  the  area  sup- 
plied by  those  posterior  roots  which  the  lesion  actually 
destroys,  and  just  above  the  level  of  the  lesion  there  is 
some  hypersesthesia  due  to  irritation. 

Referred  pains. — ^In  diseases  of  the  various  viscera, 
the  pain  to  which  they  give  rise  is  frequently  "  referred  " 
to  a  part  of  the  body  which  seemingly  has  no  direct 
connection  with  the  seat  of  the  disease.  Thus  the  head- 
ache from  eye-strain  may  be  situated  over  the  forehead, 
and  pain  between  the  shoulders  is  a  common  accom- 
paniment of  gastric  disorders. 

The  distribution  of  referred  pains  has  been  especially 
investigated  by  Head,  who  has  constructed  a  scheme  of 
intramedullary  segmentation  based  on  the  hypersensi- 
tive areas  which  result  from  irritation  of  viscera. 

The  explanation  is  that  the  impulses  which  travel 
up  to  the  cord  from  the  diseased  organs  by  the  sympa- 
thetic fibres  are  reflected  outwards  along  the  sensory 
roots  of  the  segment  at  which  they  happen  to  enter, 
and  so  the  patient  refers  the  pain  to  the  area  of  distri- 
bution of  these  roots,  and  not  to  the  viscus  from  which 
the  abnormal  impulses  originally  arise. 

Ligat|  has  applied  this  principle  to  the  diagnosis  of 
acute  abdominal  conditions,  and  finds  that  by  observing 
the  hypersensitiveness  of  certain  areas  of  the  abdominal 
*  I.e.  the  power  to  respond  to  the  compass  test, 
f  Hunterian  Lecture,  May,  1919. 


22     DISEASES   OF   THE  NERVOUS  SYSTEM 

waJl  when  tlio  .skiii  is  gently  pinclied  iij),  evidonce  can 
be  obtained  as  to  the  particular  visciis  which  is  the  seat 
of  the  disease. 

4.  Sensory  Neurones  in  the  Brain 
a.  from  medullary  nuclei  to  optic  thalamus 

In  the  previous  sections  an  outline  has  been  given  of 
the  sensory  neurones  of  the  cord  and  peripheral  system, 
and  of  the  courses  which  the  various  impulses  follow. 
The  long  fibres  of  the  posterior  columns  were  traced  up  to 
the  gracilis  and  cuneate  nuclei  in  the  medulla,  and  the 
various  connections  of  the  shorter  ones  were  indicated. 

At  the  cuneate  and  gracilis  nuclei  a  second  relay  of 
neurones  commences,  and,  after  decussating  with  their 
corresponding  fellows  of  the  opposite  side,  the  fibres 
pass  upwards  through  the  posterior  third  of  the  posterior 
limb  of  the  internal  capsule  and  terminate  in  the  optic 
thalamus. 

After  their  decussation  they  are  joined  in  their  upward 
course  by  (1)  fibres  conveying  sensations  which  have 
already  crossed  low^er  in  the  cord,  and  (2)  others  convey- 
ing sensations  from  the  head ;  and  the  combined  bundle, 
as  it  ascends  tow^ards  the  optic  thalamus,  is  known  as 
the  7nedian  fillet.  It  has  already  been  mentioned  that 
the  impulses  which  pass  up  the  long  fibres  of  the  posterior 
columns  are  those  concerned  chiefly  with  recognition  of 
posture,  spatial  discrimination,  and  vibration,  while  those 
for  tactile,  pain,  and  temperature  sensibility  crossed 
earlier  in  their  course  and  passed  up  the  opposite  side  of 
the  cord.  It  will  thus  be  seen  that,  after  the  impulses 
that  have  passed  up  the  posterior  columns  leave  the 
medullary  nuclei  and  cross,  they  again  meet  the  impulses 
that  have  crossed  lower  down,  so  that  finally  all  are 
collected  together  in  the  median  fillet.  The  fibres, 
however,  still  maintain  a  definite  grouping,  by  which 
in  certain  forms  of  disease  one  set  may  be  affected  to  the 
exclusion  of  another ;  and  the  investigations  of  Henry 
Head  and  Gordon  Holmes*  show  that  at  the  medullary 

''11  Klin,   voL  xxxiv,,    IDII. 


SENSORY   NEURONES    IN  THE   BRAIN     23 

nuclei  tlic  impulses  for  the  recognition  of  posture  leave 
those  for  spatial  discrimination.  As  far  as  the  medullary 
nuclei  they  have  travelled  together  up  the  posterior 
columns,  but  above  the  nuclei  it  is  possible  to  find  one 
set  lost  independently  of  the  other.  These  observers 
also  state  that  it  seems  that  the  impulses  underlying 
the  power  of  localizing  a  spot  touched  become  detached 
in  the  brain  stem  from  the  other  impulses  of  tactile 
sensation  with  which  they  have  been  associated  in  the 
periphery  and  cord. 

B.    FROM    OPTIC    THALAMUS    TO    CORTEX 

At  the  optic  thalamus  another  relay  of  sensory 
neurones  commences,  and  their  fibres  radiate  to  the 
cortex,  to  which  they  conduct  the  impulses. 

Present  knowledge  of  the  grouping  of  the  different 
impulses  as  they  pass  from  the  thalamus  to  the  cortex  is 
largely  due  to  the  investigations  of  Head  and  Holmes, 
who  conclude  that  the  impulses  are  grouped  into  those 
representing — (1)  recognition  of  posture  and  passive 
movement,  (2)  certain  tactile  impulses,  (3)  those  on  which 
the  appreciation  of  two  points  depends  (compass  test), 
(4)  those  by  which  the  localization  of  a  stimulated  spot 
is  recognized,  (5)  temperature  sensation. 

C.  SENSATION  AND  THE  CEREBRAL  CORTEX. 

It  has  further  been  shown  by  Head  *  that  the 
sensory  activity  of  the  cortex  is  not  concerned  with 
crude  recognition  of  touch,  pain,  heat  and  cold,  but 
that  it  exercises  discriminative  faculties  as  regards  (a) 
recognition  of  spatial  relations,  (b)  graduated  response 
to  stimuli  of  different  intensity,  (c)  appreciation  of 
similarity  and  difference  in  external  objects  brought 
into  contact  with  the  surface  of  the  body.  In  cortical 
lesions  these  three  aspects  of  sensation  may  be  dis- 
sociated from  one  another,  and  one  that  is  most  fre- 
quently disturbed  is  that  of  knowledge  of  spatial  relations 
as  shown  by  inability  to  recognize  passive  movements  of 
the  affected  part,  which  symptom  for  clinical  purposes 

*  Brain,  vol.  xli.,  pt.  2,  1918. 


24     DISEASES   OF   THE   NEEVOUS   SYSTEM 

may  be  considered  as  one  of  the  leading  signs  in  sensory 
cortical  disease.  The  localization  of  these  cortical 
sensory  functions  is,  according  to  Head,  in  the  pre-  and 
post-central  convolutions,  the  anterior  part  of  the 
su2:)erior  j^arietal  lobule,  and  the  angular  gyri.  An 
anatomical  lesion  in  these  areas  uj^sets  the  orderly 
sequence  of  physiological  processes,  which  is  shown  by 
a  disturbance  of  the  psychical  acts  of  sensation. 


CHAPTER   111 

THE     REFLEXES 

A  REFLEX  act  consists  of  a  contraction  of  a  muscle  or 
group  of  muscles  in  response  to  a  sensory  stimulus,  and, 
as  the  name  implies,  the  movement  is  the  result  of  an 
impulse  which  has  entered  the  nervous  system  and  then 
been  "  reflected  "  out  again.  A  simple  reflex  act  is  the 
most  elementary  example  of  a  coordinated  movement. 
In  their  most  primitive  forms  reflexes  always  respond 
to  the  same  stimulus  in  the  same  manner,  i.e.  their 
movements  are  stereotyped  and  incapable  of  voluntary 
modification ;  but  with  the  development  of  animals  of 
higher  type  the  more  primitive  reactions  become  con- 
trolled by  levels  of  higher  function,  so  that,  in  many 
cases,  some  choice  of  reaction  eventually  becomes 
possible  and  the  reflex  movement  can  be  modified  to  a 
varying  extent  according  to  circumstances.  When 
the  influence  of  the  higher  levels  is  abolished  by  accident 
or  disease  the  reflexes  below  are  released  from  this  higher 
control  and  tend  to  return  again  to  a  more  primitive 
state.  For  instance,  a  result  of  removal  of  one  of  the 
highest  controls  is  seen  in  the  "  extensor  response  "  of 
the  plantar  reflex,  which  is  part  of  a  more  widely  spread 
primitive  movement  by  which  the  limb  is  mthdrawn 
from  harm.  In  a  comj^lete  section  of  the  cord  this 
general  flexion  movement  of  the  limb  usually  forms 
but  one  part  of  a  "mass  reflex"  (Riddoch)  which  in- 
cludes involuntary  evacuation  of  bladder  and  rectum 
{see  p.  36). 

In  a  simple  spinal  reflex  the  impulse  travels  along  the 
sensory  nerves  to  the  spinal  cord,  which  it  enters  through 
the  posterior  roots,  and  then,  passing  through  the  grey 
matter  of  the  cord,  reaches  the  motor  cells  of  the  anterior 

25 


2G     DISEASES   OF   THE   NERVOUS   SYSTEM 

cornuti.,  from  which  impulses  pass  out  to  the  muscles  and 
give  rise  to  movements. 

The  parts  necessary  for  the  production  of  such  a  reflex 
act  are  (1)  a  specialized  end  organ  from  which  the  sensory 
ingoing  impulse  originates,  and  which  is  known  as  the 
receptor  ;  (2)  the  nerve  fibres  forming  the  paths  inw^ards 
and  outwards  along  which  the  impulses  pass,  known  as 
the  conductor  ;  (3)  the  end  organ,  known  as  the  elector, 
through  which  the  reflected  impulses  accomplish  their 
result. 

The  conductor,  even  in  the  simplest  reflex,  is  com- 
posed of  at  least  two  neurones,  and  since  the  neurones,  as 
w^e  have  already  seen,  are  independent  units,  there  is  a 
junction  at  which  the  impulses  are  transferred  from  the 
ingoing  to  the  outgoing  neurone.  This  junction  is  termed 
the  synapse,  and  there  is  some  reason  for  believing  that 
it  is  at  this  part  of  the  arc  that  variations  in  the  resistance 
to  impulses  take  place,  the  direction  along  which  they 
shall  travel  being  thus  determined.  It  is  probable  that 
no  reflex  act  in  man  is  really  so  simple  as  the  one  de- 
scribed above,  for  the  collateral  branches  of  the  con- 
ductors communicate  with  others  to  form  a  chain  far 
more  complicated  than  that  of  two  neurones. 

It  is  obvious,  from  what  has  just  been  said,  that  for  a 
reflex  act  to  take  place,  the  different  parts  of  the  arc 
must  be  intact,  and  it  follows,  therefore,  that  we  have  in 
testing  the  reflexes  a  valuable  method  of  arriving  at  some 
idea  of  the  integrity  or  otherwise  of  the  parts  of  the  arc 
through  which  the  impulses  travel.  The  behaviour  of  a 
reflex,  however,  does  not  depend  solely  upon  the  con- 
ductivity of  the  elements  that  make  ujj  the  arc,  for,  as 
already  stated,  in  normal  circumstances  a  considerable 
degree  of  modification  is  exercised  by  impulses  which 
arrive  at  the  reflex  arc  from  more  distant  parts  of  the 
nervous  system. 

On  the  deep  or  tendon  reflexes  this  modification  from 
a  distance  is  mainly  one  of  inhibition,  and  when,  for  any 
reason,  the  controlling  influence  is  removed,  the  reflexes 
usually  become  exaggerated,  e.g.  exaggeration  of  the 


EEFLEXES  27 

knee-jerk  in  disease  of  tiie  pyramidal  tracts  of  the 
cord. 

On  the  superficial  reflexes  the  impulses  from  the  higher 
centres  have  somewhat  different  effects.  In  the  case 
of  the  plantar  reflex,  removal  of  the  higher  controlling 
influences  not  only  causes  a  more  widely-spread  resj)onse, 
but,  as  will  be  seen  below,  also  changes  the  type  of  the 
reaction  from  one  of  flexion  of  the  great  toe  into  one  of 
extension — the  "  extensor  response  ;  "  while  in  the  case 
of  the  superficial  abdominal  reflexes,  removal  of  influence 
from  the  higher  centres  is  often  associated  with  diminu- 
tion or  disappearance  of  the  reflex,  which  is  the  op|)osite 
effect  to  that  produced  on  the  deep  reflexes.  Thus,  in  a 
case  of  hemiplegia,  due,  say,  to  a  haemorrhage  in  the 
region  of  the  internal  capsule,  one  would  expect  to  find 
on  the  paralysed  side  the  tendon  reflexes  of  the  arm 
and  leg  exaggerated,  the  superficial  abdominal  reflexes 
diminished  or  absent,  and  the  plantar  reflex  brisk  and 
of  the  extensor  type. 

For  clinical  purposes  reflexes  may  be  divided  into 
the  following  classes  : — 

Superficial  reflexes,  obtained  by  stimulating 
superficial  nerves  of  the  skin. 

Deep  reflexes,  produced  by  striking  certain  muscles 
or  tendons. 

Visceral  reflexes,  as  those  of  swallowing  and 
micturition. 

Bone  reflexes,  obtained  by  percussing  various 
bony  points  in  the  upper  and  lower  extremity. 

The  principal  superficial  reflexes,  with  their 
approximate  segmentation,  are  the  following  : — 

Plantar  reflex,  movement  of  foot  and  leg  produced 
by  stimulating  sole  of  foot.     S.,  1,  2,  3. 

Superficial  anal  reflex  (3rd  and  4th  sacral) ;  this 
may  be  obtained  by  stimulating  the  skin  of  the  perineum. 

Bulho-cavernous  reflex ;  this  reflex,  in  which  a  con- 
traction of  the  bulbous  part  of  the  urethra  can  be  felt 
on  stimulating  the  glans  penis,  depends  on  the  3rd  and 


28     DISEASES   OF   THE   NERVOUS  SYSTEM 

4th  sacral  segments.  The  reflex  is  frequently  lost  in 
locomotor  ataxy. 

Gluteal  reflex,  contraction  of  gluteus  on  stimulating 
buttock.     L.,  4  and  5. 

Cremasteric  reflex,  retraction  of  testicle  on  stimu- 
lating inner  and  upper  aspect  of  thigh.     L.,  1  and  2. 

Abdominal  reflex,  contraction  of  abdominal  muscles 
on  stimulating  side  of  abdomen.     D.,  8,  9,  10,  11,  12. 

Epigastric  reflex,  contraction  of  epigastrium  on 
stimulating  lower  part  of  side  of  thorax.     D,,  4,  5,  6,  7. 

Scapular  reflex,  contraction  of  scapular  muscles  on 
stimulating  skin  in  region  of  scapula.  C,  5,  6,  7,  8,  and 
D.,  1. 

Palate  reflex,  contraction  of  soft  palate  when  touched. 

Pharyngeal  reflex,  contraction  on  stimulating  posterior 
wall  of  pharynx. 

Conjunctival  reflex,  closure  of  eye  on  touching  the 
conjunctiva. 

The  ease  with  which  these  superficial  reflexes  can  be 
obtained  in  healthy  people  varies  considerably,  especially 
as  regards  the  local  conditions.  The  plantar  reflex,  for 
instance,  may  be  unobtainable  when  the  sole  of  the  foot 
is  cold  or  damp,  and  the  abdominal  reflexes  will  not 
react  with  the  same  briskness  in  an  elderly  person  as  in  a 
child.  The  degree  of  importance  to  be  attached  to  the 
reactions  in  an  individual  case  must,  of  course,  be  left 
to  the  judgment  of  the  examining  physician,  but  from 
general  observations  inferences  can  often  be  drawn  con- 
cerning (1)  the  state  of  the  fibres  of  the  arc  on  which 
the  particular  reflex  depends,  and  (2)  the  state  of  the 
upper  levels  of  the  nervous  system,  changes  in  which,  as 
we  have  seen,  often  modify  the  reaction  of  the  reflexes 
below. 

Thus,  the  presence  of  a  superficial  reflex  indicates  the 
integrity  of  the  arc  through  which  it  is  produced,  and  so 
affords  information  which  is  frequently  of  use  in  localizing 
the  level  of  disease  in  the  cord. 

Loss  of  these  reflexes,  on  the  other  hand,  does  not 
necessarily  mean  that  the  arc  is  broken,  for,  in  addition 


PLANTAR   REFLEX 


29 


to  this  possibility,  the  loss  may  depend  upon  changes  in 
the  upper  motor  neurones,  as  when  the  abdominal  reflexes 
are  lost  on  the  paralysed  side  in  cases  of  hemiplegia,  or 
when  local  conditions  of  the  skin  impair  the  sensitiveness 
of  the  receptors. 

The  plantar  reflex. — This  reflex  comprises  those  move- 
ments of  the  toes,  foot,  and  leg  which  take  place  when 
the  sole  of  the  foot  is  stimulated. 


Fig.  4. 


-Position  of  the  "big  toe  in  the  "  extensor 
response  "  (Babinski's  sign). 


When  the  sole  is  stimulated  in  the  case  of  an  infant 
the  movement  of  the  big  toe — which  for  clinical  purposes 
is  the  essential  part  of  the  movement  to  which  the 
attention  should  be  directed — takes  place  in  an  upward 
direction,  i.e.  the  toe  is  extended  and  gives  rise  to  what 
is  generally  known  as  an  "  extensor  resj)onse,"  or 
Babinski's  sign  (Fig.  4).  About  the  time  the  infant 
begins  to  walk  this  movement  of  extension  is  replaced  by 


30     DISEASES   OF   THE  NERVOUS   SYSTEM 

one  of  flexion  ;  the  toe  now  bends  downwards  instead 
of  uj) wards,  and  is  said  to  give  a  "  flexor  response." 
This  flexor  response  persists  during  the  remainder  of  life 
unless  any  interruption  of  an  organic  nature  takes  place 
in  the  conductivity  of  the  upper  motor  neurone,  in  which 
case  the  movement  returns  to  the  original  "  extensor 
response."  Therefore,  from  the  clinical  point  of  view, 
an  extensor  response,  except  in  the  case  of  an  infant,  indi- 
cates the  presence  of  a  lesion  of  the  upper  motor  neurone. 

The  best  method  of  eliciting  the  reflex  is  to  draw 
the  thumb-nail,  the  head  of  a  pin,  or  some  similar  object 
rather  firmly  along  the  sole  of  the  foot ;  in  some  cases 
the  outer,  and  in  others  the  inner,  border  of  the  sole 
gives  the  best  results. 

Oppenheim  has  shown  that  the  extensor  response  may 
sometimes  be  obtained  by  stroking  the  inner  side  of  the 
leg,  and  a  similar  reaction  can  also  occasionally  be  brought 
about  by  compressing  the  muscles  of  the  calf  (Gordon). 

To  constitute  a  satisfactory  reaction,  the  big  toe  should 
move  slowly  and  decisively  up  or  down.  If,  as  often 
happens,  a  brisk  movement  of  the  foot  and  leg  leads  to 
confusion,  a  lighter  stimulation  will,  perhaps,  bring  out 
the  toe  movement  without  that  of  the  foot,  or  the  move- 
ment of  the  latter  may  be  controlled  by  the  hand. 

If  the  reflex  is  difficult  to  obtain  or  uncertain  in 
direction,  it  is  wefl  to  test  it  when  the  leg  is  loosely  flexed 
and  also  when  it  is  extended,  for  a  reaction  is  sometimes 
better  obtained  in  the  one  position  and  sometimes  in  the 
other.  Damp  or  cold  feet,  as  already  mentioned,  make 
the  reflex  difficult  to  obtain,  and  may  lead  to  an  erroneous 
conclusion  that  it  is  absent. 

Value  in  diagnosis. — A  definite  "  extensor  response," 
after  infancy,  signifies  the  presence  of  some  modifica- 
tion of  conductivity  in  the  pyramidal  tracts,  and 
experience  shows  that  the  obtaining  of  this  extensor 
response  warrants  the  inference  that  the  interference 
is  one  due  to  organic  disease,  as  distinguished  from 
the  functional  disturbance  of  hysteria,  neurasthenia, 
and  allied  neuroses,- 


DEEP   KEFLEXES  31 

Among  the  cases  in  which  the  extensor  response  is  of 
great  value  are  undoubtedly  those  of  early  disseminated 
sclerosis,  in  which  the  patient  complains  of  indefinite 
symptoms,  combined,  perhaps,  with  exaggerated  knee- 
jerks  and  an  abortive  ankle-clonus,  all  of  which  would 
still  be  compatible  with  a  diagnosis  of  functional  dis- 
turbance. 

Absence  of  all  reflex  movement  whatever  is  sugges- 
tive of  functional  disease  if  other  symptoms  also  point 
in  this  direction,  but  the  same  reliance  cannot  be  placed 
on  a  negative  as  on  a  positive  result,  and  therefore,  while 
an  extensor  response  indicates  organic  disease,  neither 
flexor  response  nor  absence  of  response  necessarily 
excludes  it. 

A  condition  known  as  the  "  crossed  plantar  reflex  " 
has  been  described  by  Byrom  Bramwell  and  others,  who 
noticed  that  in  some  cases  of  hemiplegia,  in  which  the 
extensor  response  was  present  on  the  paralysed  side, 
stimulation  of  the  sole  of  the  foot  on  the  non-paralysed 
side  was  not  only  accompanied  by  flexor  response  on 
that  side,  but  also  by  a  flexor  response  of  the  toe  on 
the  opposite,  i.e.  the  paralysed  side. 

The  princii3al  deep  or  tendon  reflexes,  with  their 
approximate  segmentation,  are — 

1.  The  jaw-jerh. — Obtained  by  sharply  tapping  the 
chin  in  a  downward  direction  when  the  mouth  is  slightly 
open.  The  reflex  is  obtained  through  the  sensory  and 
motor  divisions  of  the  trigeminal  nerve. 

2.  The  supinator  /er^.— Obtained  by  tapping  the  lower 
extremity  of  the  radius  with  the  forearm  in  a  position 
midway  between  pronation  and  supination.  The  re- 
sulting movement  is  one  of  flexion  of  the  forearm  on 
the  arm,  in  which  the  biceps,  brachialis  anticus,  and 
supinator  iongus  take  part ;  it  corresponds  to  the  5th 
cervical  segment  of  the  cord. 

3.  The  pronator  jerk. — Obtained  by  striking  the  lower 
end  of  the  radius  on  its  antero-internal  aspect.  A  reflex 
movement  of  pronation  results  which  corresponds  to  the 
level  of  the  6th  cervical  segment. 


32     DISEASES   OF   THE  NERVOUS   SYSTEM 

4.  The  triceps  jerk. — Obtained  by  tapping  the  tendon 
of  the  triceps.  A  movement  of  extension  takes  place, 
and  the  level  of  the  reflex  corresponds  to  the  6th  and 
7th  cervical  segments. 

5.  The  knee-jerk  (2nd,  3rd,  and  4th  lumbar  segments). 
• — Full  particulars  of  this  reflex  are  given  below. 

6.  The  tendo-Achillis  jerk. — Obtained  by  tapping  the 
Achillis  tendon.  The  patient  should  kneel  in  a  chair 
with  the  feet  over  the  edge,  in  which  position  a  smart  tap 
on  the  tendon  is  followed  by  a  jerk  of  the  foot.  It 
corresponds  to  the  1st  sacral  segment. 

These  reflexes  are  generally  exaggerated  when  the 
pyramidal  tracts  above  their  level  are  diseased,  while 
they  are  diminished  or  lost  when  the  normal  inhibitory 
influence  of  the  pyramidal  tracts  is  increased  or  when 
the  continuity  of  the  reflex  arc  is  interrupted.  Their 
reaction  in  different  circumstances  may  be  exemplified 
by  a  detailed  consideration  of  the  knee-jerk. 

The  knee-jerk.— This  reflex  is  obtained  by  sharply 
striking  the  tendon  of  the  quadriceps  extensor  just  below 
the  patella,  care  being  taken  that  all  the  surrounding 
muscles  are  relaxed. 

In  doubtful  cases  relaxation  is  best  ensured  if  the 
patient  clasps  his  hands  together  and  pulls  one  against 
the  other,  his  eyes  being  meanwhile  raised  towards  the 
ceiling.  This  action,  known  as  Jendrassik's  method  of 
reinforcement,  occupies  the  attention  and  tends  to  re- 
move any  involuntary  stiffening  of  the  muscles. 

Absence  of  the  knee-jerk  indicates  organic  disease, 
as  also  does  inequality,  but  an  increase  may  be  found 
in  functional  as  well  as  in  organic  disturbances. 

The  reflex  arc  for  the  knee-jerk  is  situated  at  the  level 
of  the  2nd,  3rd,  and  4th  lumbar  roots,  and  anything  which 
impairs  the  conductivity  of  the  elements  of  this  arc  tends 
to  diminish  the  reflex.  Thus  in  peripheral  neuritis,  where 
the  mixed  nerve  is  at  fault,  the  afferent  impulses  are 
blocked  by  the  inflamed  or  degenerated  peripheral  sen- 
sory nerves,  and  even  if  they  reach  the  cord  the  chances 
are  that  the  motor  fibres  are  unable  to  conduct  them 


KNEE-JERKS  33 

outwards.  In  locomotor  ataxy  the  impulses  are  arrested 
at  the  posterior  roots,  while  in  poliomyelitis  (of  the  lumbar 
region)  the  break  occurs  at  the  cells  of  the  anterior  horns. 
Impulses  passing  down  the  pyramidal  tracts  are  con- 
stantly inhibiting  the  knee-jerks,  so  that  to  make  the 
mechanism  complete  it  is  necessary  to  consider  these 
fibres  in  connection  with  those  of  the  lower  arc* 

An  interruption  in  the  continuity  of  the  pyramidal 
fibres  is  accompanied  by  removal  of  the  normal  inhibi- 
tion, and  thus  the  knee-jerk  is  exaggerated,  e.g.  in  lateral 
sclerosis,  transverse  myelitis  (above  the  lumbar  region), 
and  other  diseases  in  which  the  pyramidal  tracts  are 
affected.  On  the  other  hand,  excitation  of  the  pyramidal 
fibres  tends  to  increase  their  inhibitory  influence,  and 
diminishes  the  knee-jerk,  as  may  be  seen  in  some  cases  of 
meningitis  and  cerebral  tumours. 

To  sum  up  :   The  knee-jerks  are  lost  (Fig.  5) — 

A.  Prom  interruption  of  the  reflex  arc. 

1.  Injuries  or  disease  of  the  lumbar  cord  causing 

destruction  of  the  "  centre." 

2.  Diseases  interrupting  impulses  to  or  from  the 

lumbar  region  of  the  cord,  e.g.  affections  of 
anterior  or  posterior  roots,  mixed  nerves,  or 
muscles. 

B.  From  inhibition  due  to  irritation  of  the  fyramidal 
■fibres i  e.g.  during  early  stages  of  cerebral  haemorrhage  and 
fracture-dislocation  of  the  spine,  and  in  some  stages  of 
meningitis.  In  many  instances,  e.g.  cerebral  haemorrhage, 
the  inhibition  is  only  temporary,  and  the  lost  or 
diminished  knee-jerk  is  soon  succeeded  by  one  that  is 
exaggerated. 

The  knee-jerks  are  increased  (Fig.  5) — 
A.  From  interruption  in  the  conductivity  of  the  pyra- 
midal fibres  above  the  lumbar  region,  whereby  the  normal 

*  Sometimes  it  happens  that  the  peripheral  ingoiag  and  outgoing 
fibres  are  extraordinarily  irritable  for  a  short  time  before  their  con- 
ductivity is  lost,  and  so  the  knee-jerks  are  occasionally  found  to  be 
increased  for  a  brief  period  during  early  stages  of  locomotor  ataxy  and 
peripheral  neuritis.     Such  cases  are,  however,  quite  exceptional. 

D 


34     DISEASES   OF  THE  NERVOUS  SYSTEM 


Fig.  5.— Diagram  to  show  causes  of  main  variations  in  the 

knee-jerk. 

In  lesions  of  the  upper  neurone  (A  and  A')  the  knee-jerk  is  usually  increased 

In  lesions  of  the  lower  neurones  or  of  the  quadriceps  extensor  muscles  (B)  the 

knee-jerk  is  usually  lost. 


inhibition  is  removed.     This   may  be  due  to   organic 
disease,  e.g.  : 

1.  Traumatic,   as  in   fracture-dislocation   of  the 
spine. 


KNEE-JERKS— VISCERAL   REFLEXES       35 

2.  Inflammatory,  as  in  transverse  myelitis. 

3.  Degenerative,  as  in  spastic  paraplegia. 

4.  The  lesion  may  be  in  the  brain,  as  in  cases  of 

hemiplegia. 

B.  In  functional  disease.  In  many  cases  of  neur- 
asthenia and  hysteria  the  knee-jerk  is  increased. 

It  will  be  observed  that  an  exaggeration  of  the  knee- 
jerk  may  accompany  both  functional  and  organic  disease, 
but  that  absence  of  the  knee-jerk  always  signifies  the 
existence  of  an  organic  lesion. 

■  The  knee-jerks  in  cases  of  complete  division  of  the 
cord. — In  cases  where  the  continuity  of  the  spinal  cord 
is  completely  divided,  by  injury  or  disease,  so  that  there 
is  a  total  interruption  of  all  impulses  at  the  level  of  the 
lesion,  the  knee-jerks,  instead  of  being  exaggerated  as 
they  are  when  the  pyramidal  tracts  alone  are  involved 
in  partial  transverse  lesions,  are  lost.  Formerly  it  was 
considered  that  this  loss  was  always  permanent,  but 
careful  observation  of  series  of  cases  in  which  the  cord 
had  been  completely  divided  by  injuries  during  the  War 
has  necessitated  some  modification  of  this  view,  for 
Riddoch*  has  shown  that  in  certain  uncomplicated 
cases  of  complete  division  of  the  cord,  after  the  initial 
shock  has  passed  ofi,  recovery  of  irritability  of  the 
isolated  portion  with  return  of  knee-jerks,  though  of 
poor  quality,  may  take  place,  provided  the  general 
health  of  the  patient  remains  good.  If,  however,  the 
tone  of  the  nervous  system  is  lowered  by  ill-health,  as 
by  septicsemic  infection  which  so  often  arises  in  these 
cases  from  cystitis,  pyelitis,  and  bedsores,  then  the  knee- 
jerks  either  do  not  return  or,  having  returned,  die  away 
again  as  the  septicaemia  develops. 

The  principal  visceral  reflexes  to  be  considered 
are  those  of  the  pharynx,  bladder,  and  rectum. 

Pharyngeal    and    laryngeal    reflexes. — When    these    re- 
flexes are  impaired  there  is  difiiculty  in  swallowing  and 
coughing.     The   food  is   apt   to   pass   into   the   larynx 
without  exciting  cough,  and  the  patient  may  be  choked, 
*  Brain,  vol.  xi.,  1918. 


36    DISEASES   OF   THE    NERVOUS   SYSTEM 

or  may  suffer  from  broncho-pneumonia  following  irri- 
tation of  the  lungs. 

Bladder  and  rectal  reflexes. — When  the  continuity  of 
the  spinal  cord  is  interrupted  above  the  lumbar  enlarge- 
ment the  reflex  arc  for  the  bladder  is  cut  off  from  all 
communication  with  the  brain.  Sensory  impulses  from 
the  full  bladder  can  no  longer  pass  upwards,  nor  can 
impulses  pass  down  to  control  the  sphincter.  The 
bladder,  therefore,  has  to  depend  upon  its  own  reflex, 
and  it  either  fills  and  discharges  automatically,  or,  as 
frequently  happens,  the  discharging  reflex  movement  is 
not  completely  carried  out,  and  the  bladder  becomes 
over-distended  and  finally  overflows,  producing  "  false  " 
incontinence.  When  the  disease  has  destroyed  the 
lumbar  enlargement  of  the  cord  the  reflex  is  lost ;  the 
walls  of  the  bladder  then  lose  their  tone  and  cannot 
retain  the  urine,  which  continually  runs  away.  Incon- 
tinence may  also  occur  from  lesions  of  the  cauda 
equina. 

The  usual  result  of  a  cord  lesion  upon  the  rectal 
sphincter  is  constipation  and  involuntary  passage  of 
faeces. 

The  mass  reflex. — The  phenomenon  known  as  the 
"  mass  reflex,"  first  described  by  Head  and  Riddoch,* 
is  a  combined  reflex  action  of  the  lower  limbs,  abdominal 
wall,  bladder,  and  sweat-glands,  which  occurs  in  certain 
cases  of  severe  injury  to  the  cord. 

Uncomplicated  cases  of  gross  injury  to  the  spinal 
cord  are  followed  by  a  period  of  shock  varying  in 
duration  from  a  few  days  to  three  weeks,  during  which 
time  there  is  complete  loss  of  function  of  the  parts  below 
the  lesion.  The  limbs  lie  motionless  and  flaccid,  and 
sensation  is  lost.  Reflex  action  in  the  isolated  section 
of  the  cord  is  almost  entirely  abolished,  and  associated 
with  this  loss  are  retention  of  urine  and  faeces  and  dry- 
ness of  the  skin.  As  the  effects  of  shock  pass  off,  this 
first  stage,  known  as  the  "  stage  of  flaccidity,"  is 
succeeded  by  a  "  stage  of  reflex  activity,"  in  which  the 

*  Jirain,  1917. 


MASS  REFLEX  37 

jiaralysed  muscles  recover  their  tone,  the  bladder  and 
rectum  commence  to  act  automatically,  and  an  area  of 
skin  varying  with  the  level  of  the  lesion  may  be^in  to 
sweat.  During  this  stage  of  reflex  activity,  in  a  well- 
defined  group  of  cases,  especially  when  the  spinal  cord 
has  been  completely  divided,  it  has  been  found  that  a 
nocuous  stimulus,  such  as  a  scratch  or  a  prick  of  any 
part  of  the  paralysed  limbs,  is  followed  by  a  flexor  spasm 
of  the  legs  at  the  hip,  knee,  and  ankle,  with  extension  of 
the  toes,  and  also,  if  the  lesion  is  high  enough,  by  a 
flexor  spasm  of  the  trunk  from  contraction  of  the 
muscles  of  the  abdominal  wall.  When  the  stimulus  is 
fairly  strong  or  applied  to  some  spot  in  the  midline  of  the 
body,  e.g.  the  region  of  the  perineum,  the  flexor  spasm 
is  often  bilateral,  there  being  a  simultaneous  contrac- 
tion of  both  legs  and  of  the  abdominal  wall.  Moreover, 
the  effects  of  the  stimulus  may  spread  to  other  parts  of 
the  cord  and  set  the  bladder  and  skin  reflexes  in  action, 
thus  producing  evacuation  of  urine  and  sweating  over  a 
skin  area  corresponding  to  the  level  of  the  lesion.  It  is 
to  this  combined  reflex  action  that  the  term  mass  reflex 
is  given.  When  the  bladder  has  begun  to  discharge 
automatically  but  is  still  unable  to  evacute  its  contents 
completely,  Riddoch*  has  shown  that  the  "  mass  reflex  " 
may  sometimes  be  utilized  to  complete  the  evacuation 
and  so  obviate  the  necessity  of  catheterization  for  the 
removal  of  the  residual  urine.  The  interval  at  which 
the  bladder  tends  to  discharge  itself  automatically  must 
be  noted  for  each  patient,  and  at  about  the  time  it  is 
due  to  evacuate  its  contents  the  "  mass  reflex "  is 
produced  by  stimulating  the  abdominal  wall  or  the 
lower  limbs,  with  the  result  that  the  reflex  activity  of  the 
bladder  is  reinforced  and  its  contents  entirely  emptied 
into  the  bottle.  Thus,  apart  from  the  avoidance  of 
catheterization,  the  risk  of  wet  beds  is  much  diminished, 
and  the  patient  is  often  able  to  carry  out  the  whole 
process  by  himself. 

Rhythmical     clonic     contractions     of     certain 
*  Lancet,  Dec.  21,  1918. 


38    DISEASES   OF   THE  NERVOUS  SYSTEM 

nuiscles  cjin  often  be  obtained  wlien  there  is  a  lesion  of 
tlie  upper  neurone. 

Ankle-clonus  is  obtained  by  suddenly  flexing  the  foot 
at  the  ankle-joint,  when,  on  keeping  up  the  pressure, 
the  foot  gives  a  series  of  jerks  against  the  hand  owing 
to  the  clonic  contraction  of  the  calf  muscles.  An  ankle- 
clonus  does  not  exist  in  health,  but  is  often  obtained 
when  the  pyramidal  tracts  are  degenerated  ;  hence  it  is 
frequently  associated  with  an  increased  knee-jerk  and 
an  extensor  plantar  response. 

Sometimes,  on  trying  to  obtain  an  ankle-clonus,  a 
few  irregular  contractions  occur  as  the  foot  is  being 
flexed,  but  cease  when  the  pressure  is  increased  and 
maintained.  These  are  known  as  "  spurious "  ankle- 
clonus  and  do  not  in  themselves  indicate  an  organic 
lesion,  although  they  do  not,  of  course,  negative  the 
possible  existence  of  one.  The  importance  of  an  abortive 
clonus  of  this  kind  must  be  judged  in  every  individual 
case  according  to  the  other  symptoms  that  may  be 
present. 

When  the  degeneration  of  the  motor  tracts  is  exten- 
sive, clonus  can  often  be  obtained  in  other  situations, 
such  as  at  the  wrist,  elbow,  and  jaw.  When  the  knee- 
jerk  is  greatly  exaggerated,  a  patellar  clonus  can  often 
be  elicited  by  suddenly  pushing  the  patella  in  a  down- 
ward direction,  and  so  starting  clonic  contractions  in  the 
quadriceps  extensor. 


CHAPTER  IV 

RIGIDITY    AND    CONTRACTURES 

When  the  conductivity  of  the  upper  motor  neurones 
is  impaired  by  injury  or  disease,  e.g.  in  cases  of  hemi- 
plegia or  myelitis,  there  ensues  a  rigidity  of  certain 
groups  of  muscles  which,  if  unchecked,  is  apt  to  be 
followed  by  permanent  contractures  of  the  affected 
limbs. 

Thus,  in  the  later  stages  of  hemiplegia  the  arm  is 
carried  in  a  characteristic  attitude  across  the  chest, 
being  drawn  close  to  the  side  by  adduction  and  flexed 
at  the  elbow,  wrist,  and  fingers,  while  the  leg  is  kept 
stiff  and  moved  as  one  piece  from  the  hip.  In  the 
cerebral  diplegia  of  children  there  may  be  sufficient 
spasm  of  the  adductors  of  the  thigh  to  cause  the  legs 
to  cross  one  another,  and  so  to  produce  the  "scissors- 
legged  "  form  of  progression  when  an  attempt  is  made 
to  walk.  (Fig.  86,  p.  331.)  In  some  cases  of  paraplegia 
from  severe  transverse  lesions  of  the  cord  the  legs 
become  fiexed  instead  of  extended. 

In  the  later  stages  of  rigidity,  degenerative  changes, 
followed  by  contraction,  take  place  in  the  fibres  of  the 
muscles  themselves  and  contribute  further  to  per- 
manent fixation  of  the  joints. 

The  cause  of  rigidity,  which  usually  comes  on  slowly 
after  lesions  of  the  upper  motor  neurones,  is  somewhat 
obscure,  but,  broadly  speaking,  it  may  be  ascribed  to  the 
unantagonized  influence  of  impulses  from  the  subsidiary 
descending  tracts,  and  especially  from  the  cerebellum 
after  impulses  from  the  cerebrum  have  been  lost.  This 
view    was    many   years    ago   presented   by   HughUngs 

39 


40     DISEASES   OF   THE  NERVOUS   SYSTEM 

Jackson,  who  propounded  the  liypothesis  that  the 
cerebrum  is  the  centre  for  changing  movements  and  the 
cerebellum  for  continuous  tonic  movements,  and  that 
when  the  impulses  from  the  cerebrum  are  cut  off,  those 
from  the  cerebellum  come  into  undue  predominance. 
The  subject  has  lately  been  more  particularly  studied 
from  the  numerous  cases  of  paraplegia  due  to  injury  of 
the  spinal  cord  which  have  been  observed  during  the 
War,  and  it  has  been  shown  that  when  the  lesion  is 
mainly  limited  to  the  crossed  pyramidal  tracts  the 
resulting  position  of  the  lower  limbs  resembles  that  of 
an  ordinary  hemiplegia^.  This  condition  is  known  as 
farajtlegia  in  extension.  If,  however,  in  addition  to  the 
interference  with  the  continuity  of  the  main  pyramidal 
tracts,  the  lesion  is  sufficiently  severe  also  to  destroy  the 
conductivity  of  the  subsidiary  motor  tracts  (rubro- 
spinal and  vestibulo-spinal),  the  tone  of  the  extensor 
muscles  completely  disappears  and,  on  stimulating  the 
limb,  the  only  reaction  that  can  be  elicited  is  a  move- 
ment of  flexion.  This  state  is  known  as  jjaraj^^Ze^ia  in 
flexion.  If  allowed  to  progress  unchecked  by  treatment 
it  may  finally  end  in  permanent  contractions  of  the 
limbs  in  the  flexed  position.  From  the  clinical  point 
of  view  it  will  thus  be  seen  that  a  "  paraplegia  in  flexion," 
with  which  is  frequently  associated  the  "  mass  reflex  " 
{see  p.  36),  is  an  indication  of  very  severe  damage,  even 
if  not  a  complete  division,  of  the  structures  of  the 
spinal  cord. 

Experimentally  the  subject  comes  under  the  heading 
of  decerebrate  rigidity,  which  Sherrington  defines  as  a 
"  condition  which  ensues  on  removal  of  the  forebrain 
by  transection  at  any  of  the  various  levels  in  the 
mesencephalon  or  the  thalamencephalon  in  its  hinder 
parts." 

The  muscles  mainly  affected  in  decerebrate  rigidity 
are  those  which,  when  the  animal  is  in  the  vertical 
position,  tend  to  counteract  the  force  of  gravity;  and 
Sherrington  points  out  that  two  separate  systems  of  motor 
innervation    can   thus    be    recognized,    one    exhibiting 


KIGIDITY  41 

"  those  transient  phases  of  heightened  reaction  which 
constitute  reflex  movements,"  while  "  the  other  main- 
tains that  steady  tonic  response  which  supplies  the 
muscular  tension  necessary  to  attitude."  Sherrington 
considers  that  Hughlings  Jackson's  view  of  the  causation 
of  these  rigidities  in  man  is  in  its  general  sense  supported 
by  the  results  of  more  recent  investigation,  for,  though 
the  forces  which  Hughlings  Jackson  attributed  to  the 
cerebellum  may  eventually  be  traced  to  the  afferent 
nerves  of  muscles  and  to  the  nerve  of  the  otic  labyrinth, 
the  existence  and  general  relationship  of  the  '*  clonic  " 
or  "  phasic  "  (Sherrington)  and  tonic  systems  remain 
the  same. 

Prevention  of  rigidity. — ^In  all  cases  it  is  most 
important  to  try  to  prevent  the  onset  of  rigidity  and 
contracture,  the  latter  of  which,  when  once  established, 
will  effectually  prevent  any  return  of  power  that  a 
partial  recovery  of  the  functions  of  the  injured  nerve 
fibres  might  otherwise  allow. 

Massage,  electricity,  and  passive  movements  are  the 
methods  upon  which  reliance  must  be  placed,  and  if 
these  are  to  be  of  real  use  they  must  be  practised  with 
regularity  and  perseverance. 

Massage  maintains  the  vitality  of  the  muscles  and 
keeps  them  supple,  the  passive  movements  prevent  the 
formation  of  adhesions  round  the  joints,  and  electricity, 
judiciously  applied,  affords  a  further  aid  to  both.  Care 
must  be  taken  to  correct  the  tendency  which  a  limb, 
e.g.  an  arm,  may  show  to  remain  in  positions  of  flexion 
and  adduction  during  the  intervals  of  treatment,  and,  if 
necessary,  it  should  be  fixed  in  a  favourable  position, 
especially  during  the  night,  by  suitable  splints  or 
supports. 

The  masseur  should  pay  particular  attention  to  the 
nutrition  and  development  of  the  weaker  groups  of 
muscles,  e.g.  the  abductors  and  extensors  of  the  arm,  so 
as  to  aid  these  as  far  as  possible  in  resisting  the  greater 
power  of  the  flexors  and  adductors. 

Persistent  treatment  on  these  principles  will  do  much 


42     DISEASES  OF  THE  NERVOUS  SYSTEM 

towards  averting  the  onset  of  rigidity,  and  will  often 
make  an  immense  amount  of  difference  to  the  ultimate 
utility  of  a  limb. 

In  lower  neurone  lesions  also,  e.g.  in  the  case  of  in- 
fantile paralysis,  deformities  are  apt  to  arise  from  the 
fibrosis  and  subsequent  contractions  of  the  paralysed 
muscles,  as  well  as  from  the  unantagonized  action  of  the 
healthy  muscles. 

Here,  again,  a  great  deal  of  trouble  may  be  obviated 
by  massage,  passive  movements,  and  electrical  treat- 
ment, care  being  taken  to  keep  the  limb  in  suitable 
positions  during  the  intervals  of  active  treatment.  The 
contractions  in  cases  of  this  class  can  often  be  improved 
by  some  operation  by  which  the  tendons  are  cut  or  length- 
ened, but  in  most  instances  it  is  necessary  also  that  the 
massage  should  be  thoroughly  carried  out  afterwards, 
otherwise  but  temporary  benefit  may  be  obtained  from 
the  operation. 

Structure  and  function  of  muscle  substance. 
— Muscle  fibres  are  composed  of  two  contractile  sub- 
stances, one  fibrillar,  which  is  easily  excited,  and  the 
other  sarcoplasmic,  of  low  contractility.  Rapidity  of 
movement  has  been  shown  to  depend  upon  the  degree 
of  differentiation  of  the  fibrillar  elements,  which  are 
considered  to  be  a  higher  form  developmentally  than 
the  sarcoplasmic  substance  (Ramsay  Hunt),  and  the 
reaction  of  muscles  in  various  circumstances  and  their 
behaviour  in  many  diseases  are  probably  influenced  by 
changes  in  the  proportionate  amount  and  quality  of 
these  substances.  For  instance,  it  has  been  suggested 
that  the  reaction  of  degeneration  is  the  result  of  dis- 
appearance of  fibrillar  substance,  accompanied  by  an 
undue  increase  of  sarcoplasmic  substance,  so  that  the 
reaction  is  that  of  sarcoplasm ;  and  the  hypothesis  has 
also  been  brought  forward  that  the  weakness  of  muscles 
in  certain  "  functional  "  disorders  may  be  due  to  a  lack 
of  proportionate  contraction  of  the  two  substances. 


CHAPTER  V 

ELECTRICAL    REACTIONS 

Normally,  contractions  of  a  muscle  can  be  obtained 
by  stimulating  its  nerve  or  the  muscle  fibres  themselves 
by  the  faradic  (interrupted)  or  galvanic  (constant) 
current. 

If,  with  the  interrupted  current,  one  pole  is  put  on 
some  indifferent  part  of  the  body,  and  the  other  on  the 
muscle  to  be  tested,  sharp  contractions  occur  in  response 
to  the  stimuli,  and,  if  the  latter  succeed  one  another 
with  sufficient  rapidity,  the  muscle  is  tetanized.  The 
maximum  effect  is  obtained  when  the  electrode  is  placed 
upon  certain  spots  known  as  motor  points.  It  is  a  matter 
of  indifference  which  pole  is  used. 

Contractions  from  the  galvanic  (constant)  current 
can  also  be  obtained  through  the  nerves  or  muscle  fibres, 
but  only  when  the  contact  is  made  or  broken. 

In  this  form  of  stimulation  there  is  choice  of  pole  to 
be  observed,  for  contraction  is  most  easily  obtained  on 
making  contact  between  the  muscle  and  the  negative 
(kathode)  pole,  as  expressed  by  the  statement  that  KCC 
is  normally  greater  than  ACC. 

When  the  communications  between  the  muscle  and 
its  corresponding  motor  cells  in  the  cord  are  broken,  as 
they  are  in  many  lower  neurone  lesions,  certain  distinctive 
modifications,  known  as  the  reaction  of  degeneration, 
are  apt  to  occur. 

The  main  features  of  this  change  are  : 

(1)  As  to  the  nerve. — Loss  of  excitability  to  both 

faradic  and  galvanic  current,   so  that  stimulation 

ceases  to  produce  any  result. 

43 


44    DISEASES   OP   THE   NERVOUS   SYSTEM 

(2)  As  to  the  muscle. — Loss  of  contraction  to 
faradic  stimulation  while  to  galvanism  the  muscle, 
though  it  may  for  a  time  be  unduly  irritable,  con- 
tracts slowly  and  in  an  abnormal  manner  to  the  two 
poles.  If  no  recovery  takes  place  the  contractility 
gradually  diminishes,  and  eventually,  when  the 
degeneration  of  the  muscle  fibres  is  complete,  no 
reaction  of  any  kind  can  be  obtained.  The  reaction 
of  degeneration  also  usually  includes  polar  changes, 
whereby  a  contraction  is  more  easily  obtained  by 
establishing  contact  with  the  positive  (anode)  than 
the  negative  (kathode)  pole  (usually  described  as 
ACC  being  greater  than  KCC),  but  experience  has 
shown  that  these  changes  are  not  so  important  for 
diagnostic  purposes  as  they  were  formerly  thought 
to  be. 

When  the  fibres  of  a  muscle  do  not  waste  uniformly, 
the  reaction  at  any  given  time  will  be  proportionate 
to  the  number  of  healthy  fibres  remaining,  and  thus 
there  will  probably  be  a  diminished  reaction  to  both 
currents,  with  no  such  qualitative  changes  as  usually 
accompany  the  reaction  of  degeneration.* 

The  use  of  condenser  discharges  for  the  testing  of 
nerves  and  muscles  has  more  recently  been  introduced. 
The  advantages  claimed  for  this  method  of  testing  are 
that  it  is  more  rapid,  more  accurate,  and  that  more 
information  can  be  obtained  from  its  use. 

Therapeutic  uses  of  electricity. — In  diseases  of 
the  nervous  system  electricity  is  chiefly  used  (1)  as  a 
general  tonic,  (2)  to  keep  up  the  vitality  of  wasting 
muscles,  (3)  to  relieve  pain. 

As  a  general  tonic  it  is  used  as  an  adjunct  to 
other  forms  of  treatment  in  hysteria,  neurasthenia, 
and  nervous  debility  of  any  kind,  and  the  choice  lies 
chiefly  between  the  interrupted,  constant,  high-fre- 
quency, and  static  forms. 

*  Other  less  common  variations  in  the  electrical  reactions  are 
alluded  to  under  the  headings  of  the  diseases  in  which  they 
occur. 


CHOICE   OF   ELECTRICAL   CURRENT       45 

The  interrupted  and  tlie  high-frequency  currents  are 
best  when  general  stimulation  is  required.  They  appear 
to  produce  a  certain  amount  of  improvement  in  nutrition, 
partly,  no  doubt,  by  increasing  the  activity  of  the  vaso- 
motor apparatus.  They  also  act  locally  as  a  counter- 
irritant. 

For  the  relief  of  pain  the  constant  current  is  mostly 
employed.  The  positive  pole  (anode)  should  be  ap- 
plied to  the  painful  part,  since  it  has  been  shown  to 
produce  a  greater  sedative  effect  than  the  negative  pole 
(kathode).  When  the  two  poles  are  kept  stationary 
(the  so-called  stabile  application)  the  current  is  less 
stimulating  than  when  the  electrode  is  moved  (the 
so-called  labile  application) ;  this  fact  therefore  enables 
a  further  modification  to  be  made  in  treating  painful 
states.  The  pains  of  neuritis,  sciatica,  and  various 
other  forms  of  peripheral  nerve  lesions  are  often  re- 
lieved by  electrical  treatment,  and  neuralgias  and  head- 
aches are  occasionally  dispersed  by  the  application  of 
high-frequency  currents. 

In  treating  disorders  of  sensation,  e.g.  the  anaesthesias 
of  hysteria,  the  application  may  often  best  be  made  by  a 
wire  brush. 

The  maintenance  of  the  nutrition  of  wasting  muscles 
is  one  of  the  most  valuable  functions  of  electricity.  For 
this  purpose  the  interrupted  current  is  generally  the  most 
useful.  An  idea  has  been  prevalent  that  the  interrupted 
current  should  be  applied  only  when  the  muscle  contracts 
to  it,  and  that  in  other  cases  preference  should  be  given 
to  the  constant  form,  but  it  was  shown  by  Lewis  Jones 
that  there  is  no  adequate  reason  for  holding  strictly  to 
this  view.  Moreover,  cases  showing  the  reaction  of 
degeneration  seem  to  have  progressed  quite  as  well  with 
the  one  as  with  the  other. 

This  question  of  the  choice  of  current  is  important, 
since  it  is  usually  far  simpler  for  the  patient  to  use  and 
maintain  a  faradic  battery. 

The  application  of  static  electricity  is  credited  with 
producing  improvement  in  general  metabolism. 


CHAPTER  VI 

THE   AUTONOMIC    SYSTEM    (SYMPATHETIC    AND 
PARASYMPATHETIC    SYSTEMS) 

The  Autonomic  (or  Vegetative)  System  forms  the 
nervous  mechanism  by  which  the  chief  functions  of  life 
are  performed  outside  the  conscious  mind. 

The  autonomic  system  consists  of  two  divisions — 
{!)  the  vertebral  sy^npathetic,  which  comprises  the  chain 
of  ganglia  situated  alongside  the  vertebral  column ;  and 
(2)  the  parasympathetic  (autonomic  system  of  Langley), 
which  takes  its  origin  from  three  principal  areas,  namely, 
the  midbrain,  the  medulla,  and  the  sacral  region.  The 
efferent  fibres  of  these  two  systems  are  mainly  distributed 
to  the  unstriped  muscle  fibres  of  the  heart  and  vessels,  to 
glands,  e.g.  the  liver  and  pancreas,  and  to  the  skin  and 
viscera,  e.g.  the  stomach,  intestine,  and  bladder.  Most 
of  these  structures  receive  a  double  nerve  supply — one 
from  the  vertebral  sympathetic  and  one  from  the 
parasympathetic  system — and  the  effects  of  physiologi- 
cal and  pharmacological  experiments  tend  to  show  that 
the  actions  of  these  two  systems  are  mutually  antago- 
nistic. Thus,  the  pupil  is  contracted  through  the 
parasympathetic  fibres  and  dilated  through  the  cer- 
vical sympathetic ;  the  heart  is  inhibited  through  the 
parasympathetic  fibres  of  the  vagus  and  accelerated 
through  the  accelerator  fibres  of  the  vertebral  sym- 
pathetic ;  the  constrictors  of  the  blood-vessels  are 
contained  in  the  vertebral  sympathetic,  and  the  dilators 
(where  they  exist)  principally  in  the  parasympathetic 
system. 

46 


AUTONOMIC   SYSTEM 


47 


Sphincter  of  iris 
ciliary  muscle 


Vessels  of  mucous 
membranes  of 
head.  Un  striped 
muscle  of  respi- 
ratory system, 
and  of  digestive 
system  down  to 
colon.  Visceral 
glands.  Heart. 
Dilator  of  iris 
and  orbital 
muscle 


Vessels,  glands,  ^ 
and  muscles  of 
skin.  Vessels  of 
alimentary  canal 
and  (?)  of  skeletal- 
muscles.  Mus- 
cles of  ureter  and 
internal  genera- 
tive organs 


Vessels  of  rectum, 
anus,  and  exter- 
nal generative 
organs.  U  n- 
striped  muscle 
of  intestine  be- 
low colon,  of 
urethra ,  bl  adder, 
and  external 
generative 
organs 


Midbrain 

autonomic 

system 


Bulbar 

autonomic 

system 


Vertebral 

sympathetic 

system 


Sacral 

autonomic 

system 


Fig.  6.— Diagram  of  the  autonomic  system  (vertebral  sympathetic 
and  parasympathetic  systems). 


48     DISEASES  OF  THE  NERVOUS  SYSTEM 

Broadly  speaking,  the  function  of  the  parasym- 
pathetic system  is  to  store  up  energy  (anabolic),  while 
that  of  the  vertebral  sympathetic  is  to  set  it  free 
(katabolic).  States  of  mind  such  as  fear,  pain,  and 
anger,  for  instance,  at  once  demand  energy  for  purposes 
of  bodily  defence,  which  energy  is  rapidly  set  in  motion 
and  results  in  such  activities  as  dilatation  of  the  pupils, 
trembling  of  limbs,  rapid  beating  of  the  heart,  and  rise 
of  blood-pressure,  all  of  which  are  associated  with  ideas 
of  self-preservation  by  attack  or  flight.  The  functions 
of  the  autonomic  system  (sympathetic  and  para- 
sympathetic) are  also  intimately  related  to  those  of  the 
ductless  glands  (endocrinic  system),  the  secretions  of 
which  regulate  the  balance  normally  existing  between 
the  two  divisions  (sympathetic  and  parasympathetic). 
It  will  thus  be  seen  how  this  balance  may  be  upset  by 
an  excessive  call  on  one  set  of  endocrinic  glands,  e.g. 
the  thyroid  or  suprarenal,  as  may  occur  through  great 
fright  or  horror,  or  from  some  primary  disorder  of  the 
glands  themselves  by  which  their  comjDcnsatory  func- 
tions become  diminished  or  lost. 

The  Parasympathetic  System 

The  efferent  fibres  of  this  system  are  medullated,  and 
those  arising  from  the  midbrain  and  medulla  run  to  their 
destination  in  company  Avith  the  cranial  nerves.  Thus, 
the  constrictors  of  the  pupil  run  with  the  third  nerve  to 
the  ciliary  ganglion  and  then  pass  on  to  supply  the 
sphincter  of  the  iris.  Secretory  fibres  for  the  salivary 
glands,  in  the  form  of  the  chorda  tympani,  and  other 
secretory  and  vaso -dilator  fibres  for  the  regions  of  the 
mouth,  nose,  and  pharynx  accompany  the  facial,  tri- 
geminal, and  glosso-pharyngeal  nerves. 

Among  the  most  important  fibres  of  the  para- 
sympathetic system  are  those  which  pass  by  way  of  the 
vagus  to  the  unstriped  muscles  of  the  heart,  blood- 
vessels, oesophagus,  and  intestines  down  to  the  level  of 
the  descending  colon,  and  also  to  the  pancreas,  liver, 
and  kidneys.     The  remainder  of  the  colon,  the  rectum, 


PLATE  4. -Diagram  of  arrangement  of  vertebral  sympathetic 

system. 


VERTEBRAL   SYMPATHETIC   SYSTEM      49 

bladder,  and  genital  organs  receive  their  supply  of  para- 
sympathetic fibres  from  the  sacral  source  which  passes 
out  from  the  cord  by  the  1st  sacral  root. 

The  Vertebral  Sympathetic  System 

The  efferent  fibres  of  the  vertebral  sympathetic 
system  arise  from  groups  of  cells  known  as  the  inter- 
medio-lateral  tract,  which  are  situated  in  the  concavity 
of  the  ventral  horns  of  the  spinal  cord,  whence  the  fibres 
pass,  as  the  white  rami-communicantes,  into  the  ganglion 
cells  at  the  side  of  the  vertebrae.  From  these  ganglion 
cells  emerge  fibres  known  as  the  grey  rami-communi- 
cantes, which  join  the  fibres  of  the  anterior  roots,  and 
thence  pass  down  the  mixed  nerves  to  their  various 
destinations.  (Plate  4.)  The  fibres  forming  the  chain 
of  the  cervical  sympathetic  arise  from  the  1st  and  2nd 
dorsal  segments  of  the  cord. 

The  vertebral  sympathetic  system  is  constantly  stimu- 
lated by  adrenaHn,  which  is  a  product  of  pigmented  cells 
known  as  chromaffin  or  chromaphil  cells,  and  is  secreted 
by  the  medullary  portion  of  the  suprarenal  body.  It  is 
probable  that  the  parasympathetic  system  is  stimulated 
by  some  substance  in  a  similar  manner.  In  this  way  a 
certain  "  tone  "  of  the  two  systems  is  established,  and  if 
for  any  reason  the  balance  between  the  two  is  upset, 
either  by  heightened  tone  of  one  or  lowered  tone  of 
the  other,  symptoms  of  disease  are  produced.  An  ex- 
cessive tone  of  the  parasympathetic  system  is  spoken  of 
as  vagotonus,  while  a  similar  condition  of  the  sympathetic 
is  known  as  sympatheticotonus. 

The  symptoms  of  disturbance  of  the  sympathetic  and 
parasympathetic  systems  are  very  numerous,  and  include 
many  of  those  of  a  so-called  functional  nature,  as,  for 
example,  flushing,  tingling,  sensations  of  giddiness,  and 
"sinking"  feelings.  Vaso-motor  symptoms  are  parti- 
cularly common,  and  may  arise  from  disturbances  in  the 
brain,  medulla,  or  spinal  cord.  For  example,  emotional 
disturbances  in  the  brain  may  cause  pallor  or  blushing ; 
vaso-motor  disturbances  of  the  medulla  give  rise  to 
£ 


50     DISEASES   OF   THE   NERVOUS   SYSTEM 

various  symptoms,  one  of  which  is  a  disorder  of  the  heart 
known  as  a  vaso-vagal  (Gowers)  or  pseudo-anginal 
attack,  characterized  by  ^palpitation,  tachycardia,  and  a 
sense  of  suffocation.  In  lesions  of  the  spinal  cord, 
anterior  roots,  or  mixed  nerves  there  may  be  vaso-motor 
symjDtoms,  as,  for  example,  is  seen  in  some  cases  of 
peripheral  neuritis  and  poliomyelitis. 

The  different  reactions  of  the  symj^athetic  and  j^ara- 
symjDathetic  systems  have  been  demonstrated  pharmaco- 
logically by  various  drugs.  Adrenalin,  for  instance,  as 
already  mentioned,  stimulates  the  vertebral  sympathetic 
and  causes  the  blood-pressure  to  rise,  the  pupils  to  dilate, 
and  the  muscles  of  the  hairs  to  contract.  It  also  stimu- 
lates the  salivary  glands  and  excites  various  areas  of 
unstriped  muscle  tissue. 

Clinically,  disturbances  of  these  systems  may  often 
be  inferred  from  signs  of  vaso-motor  instability,  as 
shown  in  the  condition  known  as  dermographism,  in 
which  a  blush  is  produced  out  of  proportion,  both  in  its 
intensity  and  duration,  to  the  stimulus  which  caused  it. 
Such  instability  is  well  seen  in  people  subject  to  urticaria, 
and  a  somewhat  similar  condition,  known  as  the  tache 
cerebrate,  is  frequently  met  with  in  cases  of  meningitis. 

In  other  instances  stimulation  of  the  skin  is  followed 
by  pallor  instead  of  redness. 

The  adaptability  of  vaso-motor  reactions  may  also  be 
tested  by  taking  the  blood-pressure  and  pulse-rate  when 
the  patient  is  in  different  positions,  such  as  lying  down 
and  standing  up. 

The  functions  of  the  cardiac  ^parasympathetic  system 
may  be  tested  by  observing  the  condition  known  as  re- 
spiratory arrhythmia  of  the  pulse  and  by  testing  the 
oculo-cardiac  reflex.  Normally  the  heart  beats  more 
raj^tidly  and  the  pulse  becomes  smaller  during  inspiration, 
while  the  reverse  takes  place  with  expiration  ;  but  if 
this  arrhythmia  becomes  very  noticeable  or  takes  place 
with  slight  res])iratory  movements  it  may  be  considered 
abnormal,  and  is  probably  to  be  looked  u^jon  as  indicating 
undue  irritability  of  the  medullary  centres.     The  oculo- 


CEEVICAL    SYMPATHETIC    TRUNK         51 

cardiac  reflex  is  the  slowing  of  the  heart  that  follows 
compression  of  the  eyeballs.  It  is  said  to  occur  in  from 
60  to  80  per  cent,  of  healthy  people  to  the  extent  of 
slowing  the  heart  six  or  eight  beats  in  the  minute. 
Excessive  slowing  ap]3ears  to  indicate  some  functional 
derangement  of  the  nervous  control  exercised  by  the 
vagus  over  the  heart,  which  is  suggestive  of  a  condition 
of  vagotonus. 

The  Cervical  Sympathetic  Trunk 

Anatomical. — The  fibres  of  the  vertebral  sympa- 
thetic system  for  the  head  and  neck  emerge  from  the 
upper  dorsal  segments  of  the  cord.  Situated  on  the 
trunk  as  it  passes  up  the  neck  are  two  and  sometimes 
three  ganglia  from  which  nerve  fibres  pass  to  their 
various  destinations. 

The  cervical  sympathetic  cord  supplies  (1)  motor 
fibres  to  the  dilator  muscle  of  the  iris,  to  the  unstriated 
fibres  in  the  upper  eyelids,  and  to  Miiller's  muscle,  which 
consists  of  a  small  collection  of  unstriped  fibres  that  pass 
across  the  spheno-maxillary  fissure  at  the  back  of  the 
orbit ;  (2)  secretory  fibres  to  the  sweat-glands  of  the 
head  and  neck  and  to  the  submaxillary  salivary  glands  ; 
(3)  vaso-motor  fibres  to  the  vessels  of  the  head  and  neck 
and  arms ;  (4)  filo-motor  fibres  to  the  skin  of  the  head 
and  neck  ,    (5)  accelerator  fibres  to  the  heart. 

The  fibres  which  cause  dilatation  of  the  pupils  arise 
in  the  medulla  and  pass  down  the  spinal  cord  to  the 
cilio-spinal  centre,  which  is  situated  in  the  cervical 
region.  They  may  thus  be  injured  before  they  leave 
the  cord.  They  emerge  from  the  cord  in  company  with 
the  anterior  roots  of  the  upper  dorsal  nerves,  and,  after 
passing  up  the  cervical  sympathetic  to  the  Gasserian 
ganglion,  reach  the  pupil  by  the  long  ciliary  branches 
of  the  5th  nerve.  .  Their  action  may  be  tested  by 
pinching  or  otherwise  stimulating  the  back  of  the 
neck,  as  a  result  of  which  dilatation  of  the  pupils 
should  occur. 

The  vaso-motor  fibres  for  the  head  and  neck  appear 


52     DISEASES   OF  THE  NERVOUS   SYSTEM 

to  leave  the  cord  at  the  level  of  the  3rd  to  6th  dorsal 
nerves. 

Paralysis  of  the  cervical  sympathetic  system 

may  arise  from  gunshot  wounds,  stabs,  enlarged  glands, 
tumours,  and  injuries  to  the  neck  of  any  other  descrip- 
tion. The  fibres  appear  sometimes  to  be  involved  in 
the  fibrosis  at  the  apex  of  the  lungs  which  occurs  in 
consequence  of  tuberculosis. 


Fig.  7.— PaTalysis  of  the  cervical  sympathetic  of  the  right 
side.  Note  the  ptosis  and  euophthalmos  of  the  right  eye  ; 
also  the  pupil  is  smaller. 

Symptoms.  Ptosis. — The  palpebral  fissure  is  narrowed 
from  paralysis  of  the  unstriped  muscle  in  the  upper 
lid,  which  causes  the  lid  to  droop  (Fig.  7). 

Enofhthalmos. — Paralysis  of  Miiller's  muscle  is  fol- 
lowed by  some  gradual  retraction  of  the  eyeball. 

Pupil. — The  pupil  on  the  affected  side  is  contracted 
from  the  unantagonized  action  of  the  constrictor  muscle, 
which  is  supplied  from  the  autonomic  system  via  the 
3rd  nerve  and  ciliary  ganglion.  It  no  longer  dilates 
when  it  is  shaded  or  when  the  cervical  sympathetic  is 
stimulated  in  the  neck,  but,  owing  to  the  integrity  of  the 
nerve  to  the  constrictor,  it  still  contracts  to  light  and 
accommodation. 

Vaso-7notor  symptoms. — Dilatation  of  cutaneous  vessels 
on  the  paralysed  side  of  the  head  and  face  sometimes 
occurs. 


AUTONOMIC  SYSTEM  53 

Anliidrosis. — ^Absence  of  sweating  on  the  side  of  the 
paralysis  may  be  present,  but  it  is  not  a  constant  symp- 
tom, and  hyperhidrosis  sometimes  occurs. 

If  the  cervical  sympathetic  is  irritated  the  signs  are 
reversed,  but  symptoms  of  irritation  are  less  frequently 
met  with  than  those  of  paralysis. 

Treatment  of  affections  of  the  autonomic 
system. — The  treatment  of  these  affections  is  not 
very  satisfactory.  The  tone  of  the  tissues  supplied  by 
the  vertebral  sympathetic  system  is  normally  kept  up 
by  a  secretion  from  the  medulla  of  the  suprarenal  body, 
so  that  adrenalin  may  be  given  in  certain  cases  where 
there  is  reason  to  think  a  lowering  of  tone  has  taken 
place. 

The  secretions  of  other  ductless  glands  besides  the 
suprarenal  body  exercise  an  influence  over  the  functions 
of  the  vertebral  sympathetic,  and  it  is  therefore  well  to 
bear  in  mind  that  disorder  of  these  glands  may  some- 
times be  the  cause  of  sympathetic  disturbance,  and  that 
in  such  cases  a  judicious  administration  of  preparations 
of,  for  example,  the  thyroid  or  pituitary  body  may 
sometimes  give  relief.  Vaso-motor  symptoms  which 
accompany  such  diseases  as  poliomyelitis  and  peripheral 
neuritis  may  sometimes  be  to  some  extent  relieved  by 
local  treatment,  e.g.  massage  and  electricity. 


CHAPTER   VII 

GENERAL   FUNCTIONS   OF   THE   BRAIN 

I.  The  Cerebrum 
The  principal  functions  of  the  cerebrum  are  to  coordinate 
impulses  received  from  the  external  world  and  to  originate 
action  accordingly.  The  cerebrum  thus  acts  as  the  organ 
of  discrimination  through  which  the  individual  is  enabled 
to  adjust  himself  to  changing  circumstances.  It  is 
especially  concerned  with  the  discrimination  of  those 
impulses  which  exercise  their  influence  from  a  distance 
through  the  mechanism  for  vision,  hearing,  and  smell, 
and  on  this  account  the  cerebrum  has  been  described 
by  Sherrington  as  "the  ganglion  of  the  distance  re- 
ceptors." 

The  general  sensory  impulses  are  conveyed  to  the 
cerebrum  and  the  motor  impulses  are  conveyed  away 
from  it  by  long  bundles  of  fibres  known  as  projection 
systems,  a  description  of  which  has  already  been  given 
(p.  5  et  seq. ).  These  different  projection  systems  are  linked 
up  with  one  another  and  with  other  parts  of  the  brain 
by  association  fibres  (Fig.  8),  and  impulses  pass  along 
these  to  reach  the  higher  centres,  where  they  form  the 
basis  of  the  psychic  processes. 

Bolton  divides  the  functions  of  the  cerebrum  into 
three  grades,  as  follows  : — 

1.  Projection  spheres,  or  regions  to  which  afferent 
sensorial  impressions  pass  from  the  parts  of  the  body 
devoted  to  the  appreciation  of  the  types  of  specialized 
sensation,  namely,  vision,  hearing,  bodily  sensibility, 
smell,  and  taste- 
s' 


CORTICAL   LOCALIZATION 


55 


2.  Centres  of  lower  association,  wliicli  lie  in  the  imme- 
diate neiglibourliood  of  each  of  the  areas  included  in  the 
first  group,  and  which  subserve  the  elaboration  of  the 
different  varieties  of  sensorial  impressions  into  simple 
perceptions  and  the  association  of  these  psychic  units 
into  higher  complexes. 

3.  The  centre  of  higher  association  and  coordination  in 
the  prefrontal  region,  which  subserves  the  grouping  of 
these  higher  complexes  into  harmonious  series  of  con- 
cepts by  means  of  voluntary  attention  and  selection. 


SHOHT   rraKSS 


UHCINATE     FAiSC. 


Fig.  8.— Diagram  of  association  fibres  of  the  cerebral  cortex. 

{Gray's  "Anatomy.") 

Cortical  localization. — The  division  of  the  cerebral 
cortex  into  areas  of  different  functional  significance  has 
been  studied  by  developmental,  clinical,  experimental, 
histological,  and  clinico-pathological  methods. 

By  the  developmental  method,  of  which  Flechsig  was 
the  principal  exponent,  the  brain  can  be  mapped  out  into 
areas  corresponding  to  the  different  periods  at  which 
myelinization  of  the  fibres  occurs,  and  by  this  means 
the  motor  and  sensory  projection  tracts  and  the  associa- 
tion fibres  can  all  be  differentiated  from  one  another 
according  to  the  time  at  which  their  myelinization  takes 
place.     (Figs.  9,  10.) 


56     DISEASES   OF  THE  NEEVOUS  SYSTEM 

The  clinical  method  consists  in  correlating  the  symp- 
toms observed  during  life  with  the  position  of  the  lesion 
found  after  death.  This  method  was  used  with  particular 
success  by  Hughlings  Jackson,  who,  from  observations 
made  on  the  distribution  of  paralysis  and  the  occurrence 
of  localized  convulsions  (Jacksonian  epilepsy),  was  able 
to  differentiate  the  functions  of  various  parts  of  the 
motor  cortex. 

The  experimental  method,  as  practised  by  Hitzig, 
Ferrier,  Horsley,  Beevor,  Sherrington,  and  others,  con- 
sists in  stimulating  the  cortex  of  an  animal  and  observing 
the  effects  on  different  parts  of  the  body.  It  has  been 
particularly  useful  in  enabling  the  motor  area  to  be 
mapped  out,  and  it  is  probable  that  the  more  recent 
results  obtain  d  by  Sherrington  and  Griinbaum  on  the 
higher  apes  are  for  all  practical  purposes  identical  with 
the  localization  of  the  corresponding  functions  in  man. 

The  results  of  histological  examinations  of  the  cortex, 
as  carried  out  by  Campbell  and  Brodman,  show  that 
the  structure  of  different  areas  differs  according  to  their 
function  ;  while  Bolton  has  made  a  valuable  contribution 
to  the  subject  in  its  pathological  aspect  by  correlating 
the  mental  .  ondition  observed  during  life  with  the 
morbid  histological  appearances  found  after  death. 

Bolton  divides  the  cortex  into  five  primary  layers  : — 

1.  Outer-fibre  lamina  or  superficial  layer. 

2.  Outer-cell  lamina  or  pyramidal  layer. 

3.  Middle-cell  lamina  or  granular  layer. 

4.  Inner-fibre  lamina  or  inner  line  of  Baillarger. 

5.  Inner-cell  lamina  or  polymorphic  layer. 
These  layers,  Bolton  states,  may  be  identified  in  all 
parts  of  the  neopallium,  but  they  differ  in  their  degrees 
of  development  in  different  cortical  areas,  as  instanced 
in  the  psycho-motor  area,  in  which  the  third  layer  is  of 
minimal  depth. 

By  combining  the  knowledge  obtained  by  these 
different  methods  it  is  possible  to  map  out  a  considerable 
area  of  the  cortex  according  to  the  functions  exercised. 

(Fig.  n.) 


CORTICAL   LOCALIZATION 


57 


PAfiJETAL    LOBE. 
aHE/iT    POSTE.R.IOR. 
ASSOai^  TIOM 


^oro-^^^;^S^z^z^o.^ 


FRONTAL 
ASSOCI/IT/Off 
canTRE. 


M/DDLE    /iSSOCIi^T/OH 
reJiVRE     OP    THE 

IHSULA 


Fig.  9. 


^oroi^ 


oft  TAC  TILE  SPfiEn^, 


/SJ"  FJZONr/iL_ 


C£AfT/i.£. 


.OLFACTORY 


^    OCClPITO     TeMPOl^^ 

Fig.  10. 


Figs.  9  and  10.— To  show  the  projection  and  association  centres 
of  Flechsig,  as  seen  on  the  external  and  internal  surfaces  of 
the  right  hemisphere.  The  dotted  areas  are  the  regions  of  the 
cortex  which  are  first  myelinized,  because  they  constitute 
the  arrival  and  departure  platforms  of  the  sensory  and  motor 
projection  systems  of  fibres.  The  remainder  of  the  cortex  is 
myelinized  at  successively  later  periods,  and  corresponds  to 
those  regions  of  the  brain  which  have  been  termed  association 
centres.    {After  Sir  F.  W.  Matt,  "Mental  Development.") 


58     DISEASES   OF   THE   NERVOUS   SYSTEM 

The  'prefrontal  lobes  are  especially  associated  with 
performance  of  the  higher  mental  functions,  and  according 
to  some  observers  the  left  lobe  is  more  important  in  this 
respect  than  the  right.  This  area  of  the  cortex  seems 
to  be  that  in  which  the  mental  processes  initiating  or  in- 
hibiting the  movements  of  the  individual  take  their  final 
form,  and  immediately  adjoining  it  posteriorly  is  the 
cortical  area  through  which  the  initiating  mental  pro- 
cesses appear  to  regulate  the  resulting  movements,  for 
when  this  part  of  the  cortex  is  diseased  on  the  left  side 
there  is  apt  to  occur  a  condition  known  as  apraxia,  the 
characteristics  of  which  are  that  the  patient  knows  what 
he  wants  to  do  but  is  unable  to  do  it,  although  there 
is  no  obvious  paralysis  of  the  part  he  desires  to  use. 
For  instance,  when  told  to  put  out  his  tongue  he  may  be 
quite  unable  to  do  so,  although  immediately  afterwards  he 
may  put  it  out  quite  naturally,  as  to  moisten  his  Hps.  In 
such  a  case  the  mental  initiation  is  apparently  good,  and 
so  also  is  the  motor  projection,  since  movements  can  be 
carried  out  for  other  purposes,  and  the  fault  therefore 
appears  to  lie  in  the  mechanism  through  which  the 
psychic  processes  initiate  the  impulses  for  movement. 
The  mechanism  for  this  process  is  situated  in  the  pos- 
terior parts  of  the  superior  and  middle  frontal  lohes  of 
the  left  side,  and  communications  are  established  with 
the  corresponding  area  of  the  right  hemisphere  by  asso- 
ciation fibres  passing  through  the  corpus  callosum. 

At  the  foot  of  the  third  frontal  convolution  on  the 
left  side,  i.e.  a  little  below  the  areas  just  described  above, 
is  Broca's  convolution,  which  is  closely  associated  with 
the  motor  mechanism  for  speech.  There  is  also  in  the 
frontal  region  an  area  concerned  with  movement  of 
the  eyes. 

Immediately  behind  the  frontal  area  comes  the 
precentral  or  ascending  frontal  convolution.  This  is 
commonly  known  as  the  motor  area,  and  its  cortex  is 
characterized  by  containing  the  large  Betz  cells  from 
which  arise  the  pyramidal  fibres  that  form  the  main 
paths  for  the  transmission  of  voluntary  motor  impulses, 


60     DISEASES  OF  THE  NERVOUS  SYSTEM 

as  described  on  p.  6.  According  to  Sherrington  and 
Griinbaum,  the  motor  function,  though  dipping  into  the 
fissure  of  Rolando,  does  not  extend,  as  formerly  thought, 
on  to  the  cortex  of  the  ascending  parietal  lobe,  which 
latter  is  apparently  entirely  concerned  with  the  reception 
of  sensory  impulses.  The  remainder  of  the  parietal  lobe 
appears  also  to  be  concerned  with  sensory  impulses. 

The  occipital  lobe  is  closely  associated  with  vision,  and 
two  areas — the  visuo-sensory,  a  lower  projection  area 
occupying  the  cortex  of  the  cuneate  lobe,  and  a  higher 
centre,  the  visuo-psychic,  occupying  the  area  of  the 
angular  gyrus — can  be  recognized. 

These  two  areas  can  be  distinguished  from  each  other 
by  their  histological  appearances  (Bolton),  and  the  angular 
gyrus  has  important  associations  in  connection  with  the 
visuo-psychic  processes  of  language  and  recognition  of 
objects,  its  destruction  resulting  in  such  conditions  as 
alexia  and  word-blindness. 

The  cortex  of  the  temporal  lobe  is  concerned  with 
hearing,  and  a  lesion  in  this  area  will  produce  deafness  of 
the  opposite  side,  usually,  however,  only  of  a  temporary 
character,  owing  to  the  cochlear  nerves  being  connected 
with  both  sides  of  the  brain.  In  the  posterior  part  of 
the  first  temporal  convolution  on  the  left  side  is  situated 
the  auditory  mechanism  of  speech,  and  disturbance  of 
this  area  gives  rise  to  various  degrees  of  auditory 
aphasia. 

Sensations  of  smell  and  taste  appear  to  be  located  in 
the  cortex  of  the  uncinate  convolution.     (Fig.  11.) 

The  optic  thalamus  consists  of  an  oval  mass  of 
grey  matter  situated  at  the  base  of  each  cerebral  hemi- 
sphere just  above  the  crura  cerebri.  The  principal 
connections  of  eacli  thalamus  are  as  follows  : — 

1.  The  posterior  part,  known  as  the  pulvinar,  is  con- 
tinuous with  the  fibres  of  the  optic  tract. 

2.  It  serves  as  a  station  for  the  reception  of  the  main 
sensory  tracts,  which,  having  passed  up  from  the  cord, 
crossed  in  the  medulla,  and  formed  the  median  fillet, 
terminate  in  the  thalamus  (.see  p.  22). 


OPTIC   THALAMUS  61 

3.  Connections  are  established  with  the  cerebral 
cortex  by  fibres  which  form  a  further  relay  of  the  sensory 
tracts,  and  also  by  the  fibres  which  conduct  impulses 
from  the  cortex  to  the  thalamus.  It  seems  to  be 
through  these  latter  that  the  cortex  exercises  control 
over  thalamic  activity. 

4.  Descending  fibres  pass  downwards  to  the  cord  in 
company  with  the  rubro-spinal  tract. 

The  functions  of  the  thalamus  have  been  investi- 
gated by  Head  and  Holmes,  who  consider  that  it  is 
the  centre  of  consciousness  for  certain  elements  of 
sensation,  and  that  it  responds  to  all  stimuli  capable  of 
evoking  either  pleasure,  discomfort,  or  consciousness  of  a 
change  in  state. 

The  characteristic  symptoms  of  a  lesion  of  the  thala- 
mus are  hemiansesthesia,  spontaneous  pains,  and  ex- 
cessive reaction  to  painful  stimuli  on  the  opposite  side 
of  the  body,  with  which  may  be  associated  involuntary 
movements  of  the  afiected  limbs.  Paralysis,  though  not 
resulting  from  a  pure  thalamic  lesion,  often  accompanies 
it  owing  to  the  proximity  of  the  motor  tract.  The 
activity  of  the  thalamus  appears  to  be  controlled  nor- 
mally by  the  cerebral  cortex,  and  when  the  communi- 
cating fibres  through  which  this  control  is  exercised 
are  destroyed,  over-activity  of  the  thalamus  results. 
This  excessive  activity  shows  itself  by  the  different 
manner  in  which  the  two  sides  of  the  body  react  to 
disagreeable  sensations,  such  as  the  prick  of  a  pin  or 
extremes  of  temperature,  and  to  emotions.  Music,  for 
instance,  may  produce  difierent  reactions  on  the  two 
sides,  and  Head  and  Holmes  mention  the  case  of  one 
patient  who  was  unable  to  go  to  his  place  of  worship 
because  he  "  could  not  stand  the  hymns  on  his  affected 
side." 

The  causation  of  the  "  spontaneous "  pains  which 
are  apt  to  occur  in  thalamic  lesions  is  generally  ascribed 
to  irritation  of  the  sensory  fibres,  but  this  explana- 
tion is  not   altogether  satisfactory,   and  Holmes*  has 

*  Trans,  Seventeenth  International  Congress  of  Medicine,  1913. 


62     DISEASES   OF   THE  NERVOUS   SYSTEM 

suggested  that  these  so-called  spontaneous  pains  are 
largely  due  to  action  of  peripheral  stimuli  on  an  over- 
sensitive thalamus  which  has  been  deprived  of  the 
control  usually  exercised  over  it  by  the  cortex.  This 
suggestion,  if  correct,  has  an  important  bearing  in 
therapeutics,  since  it  should  be  possible  to  relieve  the 
pains  by  diminishing  as  far  as  possible  the  peripheral 
stimuli  that  are  likely  to  cause  them.  The  origin  of  the 
involuntary  movements  which  may  be  associated  with 
thalamic  disease  is  not  quite  clear,  but  they  are  thought 
to  be  due  to  disturbance  of  the  functions  of  the  red 
nucleus  from  which  arises  the  rubro-spinal  tract. 

Important  investigations  on  the  higher  functions  of 
the  nervous  system  as  a  mechanism  for  the  adaptation 
of  the  organism  to  changing  external  circumstances  have 
been  made  by  Pawlow.* 

In  his  experimental  work  Pawlow  observed  that  if 
external  stimuli  are  applied  simultaneously  with  the  in- 
troduction of  some  acid  substance  into  a  dog's  mouth,  such 
external  stimuli  become  in  the  course  of  time  intimately 
connected  with  the  salivary  reaction  which  the  acid  sets 
up  in  the  dog's  mouth,  i.e.  a  new  reflex  has  been  formed, 
the  afferent  impulses  of  which  are  produced  by  the  ex- 
ternal stimuli  mentioned  above. 

To  this  newly  formed  type  of  reaction  Pawlow  gives 
the  term  conditional  reflex,  because  of  the  number  of 
conditions  on  which  its  formation  depends.  In  order  to 
produce  such  a  conditional  reflex  it  is  essential  that  the 
indifferent  stimulus  should  be  applied  synchronously 
with  the  introduction  of  the  food  or  acid  into  the  mouth, 
when,  after  a  few  sittings,  it  will  be  found  that  the 
stimulus  has  by  itself  become  capable  of  arousing  a 
secretion  of  saliva,  and  a  "  conditional  reflex  "  has  thus 
become  formed.  Pawlow  concludes  that  the  cerebrum 
is  the  organ  by  which  sensations  are  analysed  and 
through  which  new  reflexes  and  new  connections  are 
constructed,  and  that  it  is  by  such  means  that  the  animal 
is  enabled  to  adjust  itself  to  the  varied  combinations  of 
*  Brit.  Med.  Journ.,  Oct.  18,  1913,  p.  973. 


CEREBELLAR   CONNECTIONS  63 

changes  that   are  continually  taking  place  in  its  sur- 
roundings. 

IL  The   Cerebellum 

The  cerebellum  consists  of  a  median  lobe,  known  as 
the  vermis,  and  of  two  lateral  lobes. 

To  the  naked  eye  each  lobe  is  seen  on  section  to  con- 
sist of  cortex,  white  matter,  and  central  nuclei,  of  which 
latter  the  largest  is  the  nucleus  dentatum,  others  being 
the  nuclei  fastigii,  giobulosus,  and  emboliformis. 

The  connections  between  the  cerebellum  and 
other  parts  of  the  central  nervous  system  are 
both  numerous  and  important. 

Among  the  principal  of  these  are — 

A.  Afferent  fibres. — 1.  Fibres  entering  the  cerebellum 
from  the  cord  by  the  direct  cerebellar  tract  through  the 
inferior  cerebellar  peduncles  and  by  the  tract  of  Gowers 
through  the  superior  peduncles.  The  origin  and  course 
of  these  fibres  have  already  been  described  (p.  18). 
Some  fibres  from  the  columns  of  Burdach  and  Goll  also 
appear  to  enter  the  cerebellum,  and  an  important  con- 
nection with  the  posterior  columns  is  established  through 
the  external  part  of  the  nucleus  of  Burdach,  known  as  the 
nucleus  of  Monakow. 

2.  Communications  from  the  nuclei  of  the  pons, 
chiefly  of  the  opposite  side.  The  fibres  establishing  this 
communication  form  the  middle  peduncles. 

B.  Efferent  fibres. — 1.  Fibres  pass  from  the  lateral  lobes 
through  the  superior  peduncles  to  the  red  nucleus  and 
optic  thalamus  of  the  opposite  side  and  also  to  the  nuclei 
of  the  pons.  Through  these  pontine  nuclei,  communi- 
cations are  established  with  the  fronto-parietal  and 
temporal  regions  of  the  cerebral  cortex. 

c.  Communications  with  the  vestibular  nucleus. — The  ves- 
tibular nerve  is  closely  associated  with  the  cerebellum 
through  connections  between  the  vestibular  nucleus, 
the  nuclei  of  the  cerebellum,  and  the  nucleus  of 
Deiters.  Communications  are  also  established  through 
the  nucleus  of  Deiters  with  the  ocular  nuclei,  as  described 
under  the  head  of  Nystagmus  (p.  93).     Fibres  likewise 


64     DISEASES  OF   THE  NERVOUS   SYSTEM 

pass  from  the  vestibular  nucleus  downwards  to  the 
cord  by  the  vestibulo-spinal  tract,  where  they  establish 
communications  with  the  cells  of  the  anterior  horns, 
and  so  form  a  mechanism  which  assists  in  maintaining 
muscle  tone. 

D.  Association  fibres. — By  means  of  association  fibres 
communications  are  established  between  different  parts 
of  the  cerebellum. 

Functions. —  The  cerebellum  is  principally  con- 
cerned with  the  maintenance  of  equilibrium  and  muscle 
tone. 

Impulses,  which  vary  according  to  the  position  of 
the  head  in  relation  to  surrounding  space,  are  conveyed 
along  the  vestibular  nerves  to  the  cerebellum,  whence 
the  movements  of  the  head  and  eyes  are  appropriately 
controlled. 

If  this  mechanism  is  disturbed,  nystagmus  and 
vertigo,  which  are  such  frequent  accompaniments  of 
disease  of  the  cerebellum,  are  produced. 

Interference  with  the  efferent  fibres,  of  which  the 
principal  are  the  vestibulo-spinal  tract  and  those  flowing 
from  the  cerebellum  to  the  optic  thalamus  and  the  red 
nucleus,  is  followed  by  loss  of  muscle  tone  (hypotonus) 
chiefly  on  the  same  side  of  the  body  as  the  lesion. 

There  may  also  be  a  difficulty  in  executing  alternating 
movements  of  a  limb,  such  as  pronation  and  supination 
of  the  arm,  with  rapidity,  and  to  this  difficulty  the  term 
adiadochohinesis  has  been  applied. 

Coordination  of  muscular  movements  (synergia)  and 
the  regulation  of  the  strength  of  nervous  impulses  pass- 
ing to  the  muscles  {eumetria)  are  carried  out  by  means 
of  the  cerebellum. 

The  most  obvious  symptom  of  cerebellar  disease  is 
ataxy.  Cerebellar  ataxy  is  most  marked  in  the  coarser 
movements  of  the  trunk  and  limbs,  with  the  result  that 
the  patient  walks  with  a  reeling  gait  that  has  been 
likened  to  that  of  a  person  endeavouring  to  maintain  his 
equilibrium  on  the  deck  of  a  rolling  ship,  to  which  there 
is   a   further   similarity  in  the  sudden   lurch  that  the 


CEREBELLAR    FUNCTIONS  65 

cerebellar  patient  occasionally  gives  to  one  side — ^usually 
towards  the  side  of  the  lesion — or  backwards  if  it  is  the 
vermis  that  is  diseased. 

In  the  arms  the  ataxy,  though  less  marked,  is  shown 
by  the  erratic  manner  in  which  an  attempt  is  made  to 
make  some  particular  movement,  such  as  placing  the 
finger  on  the  tip  of  the  nose — an  attempt  that  is  also 
often  accompanied  by  tremor  of  the  limb. 

Babinski  has  pointed  out  that  the  movements  in  cere- 
bellar disease  are  in  excess  of  what  is  required  to  accom- 
plish their  object,  and  that  when  an  attempt  is  made  to 
place  the  finger  on  the  tip  of  the  nose  the  movement 
does  not  cease  at  the  right  moment  but  is  prolonged  in 
such  a  way  that  the  finger  passes  over  the  nose  and  strikes 
the  jaw.  In  the  same  way  a  line  drawn  on  a  sheet  of 
paper  is  prolonged  beyond  the  point  at  which  it  was 
desired  that  it  should  stop.  The  ataxy  of  cerebellar 
disease  is  modified  but  little  by  closing  the  eyes;  in 
this  particular  it  differs  from  that  of  tabes. 

To  sum  up,  it  may  be  said  that  the  cerebellum  forms 
the  centre  on  which  the  impulses  of  deep  sensibility 
converge,  and  from  which  impulses  pass  to  regulate  the 
tone  of  the  muscles  and  the  smoothness  of  their  action ; 
and  thus,  in  the  words  of  Sherrington,  it  may  be  said 
that  "  the  cerebellum  is  the  head  ganglion  of  the  proprio- 
ceptive system." 


CHAPTER   VIII 

EXAMINATION   OF    THE   HIGHER   FUNCTIONS 
OF   THE   NERVOUS    SYSTEM 

Where  the  symptoms  complained  of  concern  the  higher 
functions  of  the  nervous  system  it  is  important  to  test  the 
integrity  of  those  parts  of  the  brain  which  underhe  these 
functions,  and  as  far  as  possible  to  obtain  some  record  of 
the  manner  in  which  they  respond. 

The  advances  which  experimental  psychology  has 
made  in  recent  years  now  render  it  possible  to  bring  many 
of  its  methods  into  common  clinical  use,  thereby  increas- 
ing the  interest  and  accuracy  of  the  diagnosis  of  some  of 
the  vaguer  forms  of  nervous  diseases  usually  classed  under 
the  heading  of  "  neuroses."  The  value  of  such  tests  lies 
in  the  correlation  of  the  results  with  all  the  other  symp- 
toms observed  in  the  case,  and  they  should  be  looked 
upon  in  the  nature  of  additional  physical  signs. 

The  subject  is  a  large  one,  and  space  allows  only  a 
brief  account  to  be  given  of  a  few  of  the  methods  which 
the  author  has  found  of  practical  value. 

Reaction  times. — Records  for  reaction  times  for 
touch,  vision,  and  hearing  may  in  appropriate  cases  be 
of  considerable  value.  The  interval  which  takes  place 
between  the  stimulus  and  the  reaction  not  only  serves 
as  a  guide  to  the  rapidity  with  which  coordination  of 
the  particular  movement  is  carried  out,  but  also  forms 
a  record  for  comparison  between  the  states  of  the 
patient  at  different  times. 

The  accompanying  diagrams  show  the  visual  re- 
action times  taken  with  the   same   instrument   under 

66 


TIME-DISCRIMINATION    TEST 


67 


precisely  similar  conditions  in  (1)  a  normal  person, 
(2)  an  early  stage  of  paralysis  agitans,  and  (3)  a  late 
stage  of  paralysis  agitans.     (Fig.  12.) 


U 


Fig.  12.— Records  of  visual  reaction  times  in  (i)  a  normal 
person,  (2)  an  early  stage  and  (3)  a  late  stage  of  paralysis 
agitans.  The  vertical  lines  at  the  foot  of  each  diagram 
are  those  of  the  time-marker,  beating  half-seconds. 


Slowness  of  movements  is  a  well-known  symptom  of 
paralysis  agitans,  and  in  Fig.  12  (2),  where  the  patient  was 
in  quite  an  early  stage  of  the  disease,  the  record  served 
as  an  additional  corroboration  of  the  diagnosis.  Distinct 
abnormalities  in  reaction  times,  provided  the  afferent  and 
efferent  paths  are  normal,  indicate  the  probability  of 
faulty  coordination  of  impulses  in  the  higher  cerebral 
centres ;  as  such,  when  taken  with  other  signs,  they  are 
suggestive  of  the  presence  of  organic  as  opposed  to 
functional  changes,  and  thus  may  give  a  certain  amount 
of  assistance  in  diagnosing  degenerations  in  their  early 
stages. 

Indications  of  fatigue  may  be  recorded  either  by 
special  instruments  or  by  timing  the  quantity  and 
estimating  the  quality  of  mental  and  physical  work  of  a 
known  standard. 

A  useful  mental  test  for  clinical  purposes  is  that  known 
as  time  discrimination,  when  given  to  those  who  are 


68     DISEASES   OF   THE  NERVOUS   SYSTEM 

accustomed  to  dealing  with   figures  and  letters.     The 
following  arrangement  of  figures  is  taken  from  Franz  :* — 


4 

3 

8 

5 

9 

3 

4 

5 

2 

4 

7 

6 

5 

8 

7 

2 

3 

6 

6 

3 

7 

6 

3 

9 

8 

6 

3 

7 

7 

9 

3 

5 

6 

4 

8 

8 

9 

4 

6 

8 

9 

4 

7 

9 

7 

8 

4 

8 

5 

9 

8 

5 

3 

2 

8 

6 

9 

8 

3 

9 

7 

2 

8 

7 

6 

4 

3 

8 

5 

8 

5 

7 

2 

5 

7 

2 

8 

2 

8 

7 

9 

4 

3 

7 

6 

2 

8 

3 

7 

9 

7 

5 

3 

5 

5 

6 

7 

9 

4 

9 

2 

2 

4 

8 

4 

2 

3 

7 

4 

5 

7 

6 

3 

9 

3 

6 

5 

5 

9 

5 

7 

2 

6 

2 

9 

9 

4 

2 

9 

7 

8 

*7 

2 

8 

3 

5 

6 

4 

7 

8 

7 

2 

4 

3 

2 

6 

6 

5 

9 

4 

5 

8 

8 

3 

2 

4 

8 

2 

6 

2 

3 

3 

4 

4 

6 

2 

5 

2 

4 

5 

2 

5 

7 

5 

4 

6 

4 

3 

3 

9 

5 

9 

6 

2 

9 

5 

6 

6 

4 

9 

6 

6 

7 

The  test  consists  in  the  patient  marking  off  every 
3  as  quickly  as  he  can,  the  result  being  judged  by  the 
rapidity  and  accuracy  with  which  this  is  done.  Some- 
where  about   forty-five   seconds    is    considered    to    be 

*"  Handbook  of  MeutalJ  Examiuaticu  Methods,"  by  Shepherd 
Ivory  Franz. 


MEMORY   TESTS  69 

the  approximately  average  time  taken  by  ordinary 
persons. 

Where  this  test  is  badly  performed  its  repetition  at  a 
later  date  often  gives  a  useful  indication  as  to  whether 
or  not  the  patient  has  made  any  improvement.  For 
instance,  a  neurasthenic  patient  who  was  accustomed  to 
deal  with  figures  in  his  daily  work  took  sixty  seconds 
over  the  test  when  first  seen,  but  after  a  course  of 
treatment,  from  which  he  derived  great  general  benefit, 
he  was  able  to  reduce  the  time  to  forty-five  seconds, 
thus  corroborating  the  fact  of  his  improvement. 

Another  useful  form  of  test  is  that  for  memory. 
Patients  are  constantly  complaining  of  loss  of  memory, 
and  it  is  therefore  important  to  be  able  to  form  some 
conclusion  as  to  the  reliability  of  their  observations, 
since  it  very  often  happens  that  what  is  described  as 
loss  of  memory  is  in  reality  due  to  feebleness  of  mental 
concentration,  and  not  to  any  disturbance  of  the  memory 
mechanism  itself.  Different  types  of  memory  may  be 
tested,  such  as  the  visual  (tested  by  saying  off  a  series 
of  letters  or  figures  after  having  looked  at  them  for 
a  few  moments),  the  auditory  (tested  by  repeating  a 
similar  series  that  have  been  heard),  and  the  associative 
(tested  by  reading  out  to  the  patient  a  list  of  words 
associated  with  one  another,  and  then  reading  out  the 
first  words  on  the  list  and  asking  him  to  recall  the 
associated  ones). 

Many  other  tests— e.g.  for  memory  of  time,  place,  and 
nam.es — can  be  applied  as  required,  and  it  is  important 
to  remember  that  the  memory  for  recent  events  may 
in  some  circumstances  be  much  weaker  than  that  for 
events  that  happened  long  ago,  as  is  frequently  met 
with  in  the]  failure  of  memory  that  commonly  occurs  in 
old  age. 

These  various  forms  of  loss  of  memory  may  be 
associated  with  very  different  conditions.  For  example, 
an  imbecile  may  possess  a  good  visual  or  auditory 
memory  and  yet  be  unable  to  associate  ideas  to  any 
appreciable    extent ;     and    the    frequency    ^vith    which 


70     DISEASES  OF  THE  NERVOUS  SYSTEM 

memory  for  recent  impressions  fails  before  that  for 
those  of  long  standing  has  just  been  mentioned.  As 
illustrating  the  interest  of  such  tests,  a  patient  com- 
plained, among  other  symptoms,  that  he  was  unable 
to  remember  the  drift  of  speeches  which  he  was  in  the 
habit  of  making,  and  on  testing  his  memory  it  was  found 
that  while  his  visual  and  auditory  memories  were  both 
good,  his  powers  of  remembering  associations  were  weak. 
Here  the  patient's  own  account  was  confirmed  by  the 
test,  and  a  more  accurate  estimation  of  the  importance 
of  the  symptom  could  thus  be  made. 

Jung's  word-association  tests  are  often  of  use 
as  an  aid  towards  discovering  the  particular  line  of 
thought  with  which  the  patient  is  troubled.  This  method 
consists  in  reading  out  a  list  of  chosen  words  to  the  patient, 
instructing  him  to  respond  by  uttering  the  first  -^^^ord 
which  the  read-out  word  suggests  to  him,  while  the 
examiner  notes  with  a  stop-watch  the  exact  time  taken 
in  making  the  association.  Jung  has  shown  that  this 
process  of  making  associations  is  concerned  with  the 
emotions  rather  than  mth  the  intellect,  and  when  one 
of  the  test  words  read  out  strikes  some  special  mental 
complex  the  emotion  thereby  aroused  is  betrayed  by 
certain  peculiarities  in  the  mode  of  reaction,  and  especially 
in  the  increased  time  taken  to  produce  it.  By  ob- 
serving the  kind  of  words  which  have  produced  abnormal 
reactions,  and  by  making  up  fresh  test  lists  to  include 
words  especially  connected  with  those  that  have  already 
aroused  emotion,  it  is  often  possible  to  derive  assistance 
in  coming  to  some  conclusion  regarding  the  complexes 
that  are  influencing  the  patient's  actions. 

Long  reaction  times  and  other  pecuHarities  show 
an  imperfect  adaptation  of  the  individual  to  the  con- 
ditions the  stimulus  words  have  called  up,  thus  in- 
dicating directions  in  which  faulty  reaction  may  take 
place.  The  method  may  be  used  also  as  a  guide  to 
progress  by  comparing  the  reaction  types  from  time  to 
time  during  the  course  of  the  illness. 

The  psycho-c|alvanic  reflex,  in  which  deflection 


PSYCHO-GALVANIC   REFLEX  71 

of  a  galvanometer  is  produced  by  emotional  states,  can 
also  be  used  to  indicate  the  presence  of  emotional 
memories. 

Degrees  of  mental  deficiency  can  be  estimated  by  the 
Binet-Simon  tests  and  their  modifications,  for  accounts 
of  which  the  reader  is  recommended  to  consult  special 
works. 


CHAPTER   IX 

PATHS   OF    INFECTION   OF   THE    CENTRAL 
NERVOUS    SYSTEM 

Apart  from  extension  by  direct  continuity  of  tissues, 
toxic    material    reaches    the    central    nervous    system 


Fig.  13.— A  lymphogenous  infection— chronic  tabes  dorsalis. 

(Orr  and  Ro%vs.) 

principally  by  two  channels — (1)  the  blood,  and  (2)  the 
lymph. 

That  infection  may  be  hsematogenous  in  origin  has 
been  long  known,   but  the  fact  that  it   may  also    be 

72 


LYMPHOGENOUS  INFECTION 


73 


lymphogenous  depends  upon  later  observations,  and 
more  especially  upon  the  researches  of  Orr  and  Rows.* 
The  experimental  work  of  these  observers  shows  that 
infection  may  be  conveyed  to  the  cord  through  the 
lymphatic  system  of  the  peripheral  nerves;  and  that 
this  also  happens  in  man  has  been  proved  by  clinical 
observations  in  which  infection  of  the  cord  has  been 
traced  by  this  path  from  such  local  conditions  as  car- 
cinoma of  the  tongue,  erysipelas  of  the  face,  bedsores  in 
juvenile  general  paralysis,  and  tuberculous  lumbar  abscess. 


Fig.  li.— Ahsematogenous  infection— Addison's  disease. 
{Orr  and  Rous.) 

The  authors  also  allude  to  a  very  important  class  of 
cases  in  which  infection  of  the  cord  sometimes  follows 
inflammation  of  the  urinary  bladder.     They  relate  an 
*  Froc.  Jtoii.  Soc.  Med.  (Neurological  Section),  19U. 


74     DISEASES  OF  THE  NERVOUS  SYSTEM 

instance  in  which  transverse  myehtis  followed  chronic 
bladder  trouble,  and  refer  to  other  instances  of  a  similar 
nature. 

From  their  clinical  observations  Orr  and  Rows 
conclude  (1)  that  the  locality  of  the  lesion  in  the  spinal 
cord  always  corresponds  to  the  nerve  supply  of  the 
infective  focus ;  (2)  that  the  degeneration  of  the  intra- 
medullary portion  of  the  spinal  roots  commences  at  the 
point  where  the  neurilemma  sheath  is  lost ;  (3)  that 
the  posterior  root  entry  zone  is  always  most  afEected ; 
(4)  that,  as  examination  of  the  extrameduUary  portion 
of  the  nerves  yielded  a  negative  result,  it  seems  correct 
to  assume  that  toxins  can  in  certain  cases  ascend  along 
the  perineural  lymphatics  without  producing  paren- 
chymatous changes  in  the  nerves. 

From  the  correlation  of  the  changes  found  after 
death  with  the  symptoms  observed  during  life  it  appears 
that  absorption  may  take  place  along  the  ascending 
lymph  paths  of  the  nerves  for  a  considerable  period 
before  the  exciting  agent  is  of  sufficient  potency  to 
cause  symptoms.  The  explanation  of  this  probably 
lies  in  the  fact  that  the  anatomical  arrangement  of 
the  structures  is  capable  of  causing  attenuation  of  the 
noxious  substances  as  they  approach  the  central  ner- 
vous system. 

The  highly  vascular  epidural  tissue  and  the  dura 
itself  form  a  barrier  to  the  spread  of  infection.  Thus  in 
the  early  stages  of  infection  the  exciting  agent  reaches 
the  cord  in  small  quantities  or  in  an  attenuated  con- 
dition and  gives  rise  to  little  disturbance,  but  should 
the  defence  mechanism  be  broken  down  acute  inflam- 
mation occurs. 

Between  the  experimental  results  of  lymphogenous 
and  hsematogenous  infection  Orr  and  Rows  make  the 
following  distinctions  : 

In  lymphogenous  infection  there  are  found  (1)  re- 
action of  the  cells  of  the  fixed  connective  tissues;  (2) 
proliferation  of  the  cells  of  the  adventitial  sheath  of  the 
veins  and  capillaries ;    (3)  the  appearance  of  scavenger 


PATHS  OF  INFECTION  75 

cells  where  the  myelin  is  disintegrated  ;  (4)  nerve-cell 
degeneration  and  neuronophage  phenomena. 

In  hcematogenous  infection  (1)  the  most  highly  de- 
veloped structures,  i.e.  the  nerve  cells,  suffer  least  of 
all.  (2)  There  is  primary  degeneration  of  the  myelin 
sheath  round  the  cord  margin  and  on  either  side  of  the 
postero-median  septum.  (3)  The  myelin  degeneration 
is  greatest  in  the  upper  part  of  the  cord.  (4)  There  is 
oedema  of  the  cord.  (5)  The  vessel  walls  are  hyaline, 
and  contain  thrombi  of  the  same  nature. 

The  difference  in  the  distribution  of  the  degenera- 
tion in  the  two  forms  of  infection  is  shown  in  the  accom- 
panying figures  (Figs.  13,  14). 

Among  inflammations  which  are  probably  of  lympho- 
genous origin  Orr  and  Rows  include  many  cases  of 
acute  and  subacute  meningomyelitis,  acute  poliomyelitis, 
tabes  dorsalis,  and  general  paralysis,  while  those  of 
hsematogenous  origin  comprise  examples  of  subacute 
non-systemic  lesions  of  the  cord  such  as  may  be  associated 
with  conditions  like  pernicious  anaemia  and  Addison's 
disease. 


CHAPTER  X 

LUMBAR  PUNCTURE 

Technique. — For  a  lumbar  puncture  the  interval 
between  the  3rd  and  4th  or  between  the  4th  and  5th 
lumbar  vertebrae  should  be  selected.  (Fig.  15.)  A  line 
joining  the  highest  points  of  the  iliac  crests  passes  through 
the  4th  lumbar  spine,  so  that  the  puncture  should  be 
made  either  above  or  below  the  line,  midway  between 
the  spinous  processes,  and  J  in.  or  rather  less  from  the 


7z"\  Vz 

♦--•■!--■'» 

® 


f$>^  ^"(7^ 


Fig.   15.— Diagram    showing  positions  suit-able  for   lumbar 
puncture.    (H.  MacCormac.) 

middle  line.  It  is  scarcely  necessary  to  say  that  the  skin, 
the  needle,  and  the  hands  of  the  operator  should  be 
thoroughly  sterilized.  The  skin  over  the  chosen  spot 
should  be  frozen,  but  in  operating  on  children  a  general 
anaesthetic  is  sometimes  necessary. 

The  needle,  which  in  the  case  of  an  adult  should 
be  about  3^  or  4  in.  long,  and  in  that  of  an  infant 
about  2|  in.,  should  have  the  cahbre  of  an  ordinary 
needle  for  the  injection  of  antitoxin. 

Position. — The  patient  should  lie  on  the  side, 
with  the  knees  drawn  up  and  the  back  arched  outwards. 

76 


LUMBAR   PUNCTURE    -  77 

When  all  is  ready,  tlie  needle  is  inserted  forwards  and 
slightly  inwards,  and  if  it  strikes  upon  bone  it  must  be 
withdrawn  a  little  and  its  direction  somewhat  changed. 
There  is  no  great  difficulty  in  discriminating  between 
bone  and  ligamentum  subflavum,  which  also  offers  some 
resistance. 

If  the  pressure  is  normal  the  fluid  flows  out  drop  by 
drop  when  the  point  of  the  needle  is  in  the  arachnoid 
space,  but  if  the  pressure  is  increased  the  flow  is  more 
forcible.  Occasionally  a  little  blood  from  some  small 
vessel  is  mixed  with  the  first  few  drops,  but  this  soon 
disappears.  Some  operators  prefer  to  use  a  syringe  as  a 
handle  to  the  needle,  but  this  is  not  necessary,  and  only 
in  exceptional  cases  should  any  suction  power  be  used 
to  withdraw  the  fluid. 

Value  in  diagnosis. — For  ordinary  purposes  of 
diagnosis  about  a  drachm  of  the  fluid  is  sufficient,  and  it 
should  be  received  into  a  sterilized  test-tube.  This  may 
be  chemically  examined,  and,  after  it  has  been  centri- 
fugalized,  slides  for  the  microscope*  and  cultivations  of 
micro-organisms  can  be  made. 

Normal  cerebro-spinal  fluid  is  quite  clear,  and  when 
examined  under  the  microscope  contains  no  cellular 
elements,  or  at  most  a  very  few  lymphocytes. 

In  meningitis  the  fluid  is  often  turbid,  though  in  the 
tuberculous  variety  it  may  remain  clear.  In  addition 
to  bacteriological  evidence  of  disease,  microscopical  exa- 
mination shows  the  presence  of  lymphocytosis,  and  in 
the  non-tuberculous  varieties,  such  as  epidemic  cerebro- 
spinal, streptococcal,  and  pneumococcal  meningitis,  poly- 
morphonuclear cells  also  are  generally  present. 

The  reaction  for  sugar,  which  can  be  obtained  from 
normal  fluid,  may  be  diminished  or  absent  in  meningitis. 

Haemorrhage  of  old  standing  into  the  cerebro-spinal 
fluid,  from  vessels  of  the  meninges  of  the  brain  or  of  the 
cord,  may  cause  a  yellowish  discoloration  of  the  fluid, 
and  in  cases  where  the  cord  is  compressed — especially 
towards  its  lower  end — the  fluid  may  show  the  charac- 
*  For  these  Jenner's  stain  or  methylene  blue  may  be  used. 


78     DISEASES   OF   THE  NERVOUS  SYSTEM 

teristics  known  as  Froin's  syndrome — yellow  discolor- 
ation, pronounced  coagulability,  and  an  increased 
number  of  lymphocytes. 

Lymphocytosis  can  often  be  demonstrated  in  the 
early  stages  of  general  paralysis  of  the  insane,  tabes, 
and  syphilitic  diseases  of  the  nervous  system.  It  has 
also  been  found  in  disseminated  sclerosis  and  anterior 
poliomyelitis. 

Undue  stress  must  not  be  laid  on  this  method  of 
diagnosis,  and  in  all  doubtful  cases  the  results  should  be 
carefully  considered  in  conjunction  with  the  other  signs 
before  a  decision  is  made. 

Value  in  therapeutics. — The  therapeutic  value 
of  lumbar  puncture,  apart  from  its  use  as  a  j)reliminary 
step  to  the  injection  of  serums  into  the  spinal  canal,  lies 
in  the  relief  of  intracranial  pressure,  though  in  most 
cases  its  efiects  are,  unfortunately,  only  transient.  It 
has,  for  instance,  been  found  useful  in  cases  of  cerebral 
tumour  and  meningitis,  and  also  in  cases  of  coma  from 
renal  disease  and  injuries  to  the  skull. 

Some  unpleasant  symptoms,  such  as  headache, 
nausea,  and  giddiness,  may  occasionally  follow  lumbar 
puncture,  and  it  is  always  advisable  for  the  patient 
to  rest  for  some  hours  after  the  operation.  Care  must 
also  be  taken  not  to  withdraw  tco  much  fluid ;  the 
amount  naturally  varies  with  the  pressure  of  the  fluid 
and  the  conditions  for  which  it  is  drawn  ofi,  but  if  this 
point  be  borne  in  mind  it  is  not  likely  that  an  excessive 
amount  will  be  withdrawn. 


SECTION  II.— DISEASES  OF  THE 
PERIPHERAL  NERVES 

CHAPTER   XI 
PARALYSIS    OF    CRANIAL    NERVES 

I.   THE  FIEST   OR   OLFACTORY  NERVE 

The  olfactory  apparatus  comprises  the  following  ele- 
ments :  (1)  The  olfactory  cells,  situated  in  the  olfactory 
mucous  membrane,  the  central  processes  of  which  are 
continuous  with  the  fibrils  of  the  olfactory  nerves  proper. 
The  latter  end  in  (2)  the  olfactory  bulbs,  by  arborizing 
round  the  termination  of  the  dendrites  of  the  so-called 
mitral  cells,  situated  more  deeply  in  the  substance  of 
the  bulb.  The  centrally  directed  mitral  axon  is  then 
conducted  along  (3)  the  olfactory  tracts,  which  are  each 
connected  by  means  of  a  mesial  and  a  lateral  root  with 
(4)  the  uncus,  the  cortical  centre  for  smell.  The  olfactory 
apparatus  thus  consists  of  a  series  of  neurones  :  the 
olfactory  cells,  the  mitral  cells  of  the  hulh,  and  the  cortical 
cells  of  the  uncinate  convolution. 

The  olfactory  nerves  proper,  about  twenty  in  num- 
ber on  each  side,  pass  through  the  cribriform  plate  of  the 
ethmoid,  and  tend  to  be  arranged  in  an  inner  and  an 
outer  group  ;  the  fibrils  of  the  former  are  distributed 
over  the  upper  part  of  the  ethmoidal  septum,  and  those 
of  the  latter  supply  the  mucous  membrane  overlying 
the  superior  turbinated  bone.  The  'peripheral  process 
of  the  olfactory  cells,  of  which  these  nerve  fibrils  are  the 
central  offshoots,  ends  on  the  surface  of  the  olfactory 
mucous  membrane,  and  is  surrounded  by  a  tuft  of  short 
filaments,  the  olfactory  hairs. 

To  test  the  sense  of  smell,  aromatic  substances, 
e.g.  cloves  and  peppermint,  should  be  applied  in  turn 

79 


80     DISEASES   OF  THE  NERVOUS   SYSTEM 

to  each  nostril,  the  other  being  meanwhile  closed.  Sub- 
stances which  stimulate  the  5th  nerve,  e.g.  ammonia, 
must  not  be  used,  otherwise  the  sensations  thereby  pro- 
duced may  be  mistaken  for  those  of  smell.  The  local 
causes  of  perversion  or  loss  of  this  sense  are  very  numer- 
ous, and  must  be  excluded  before  the  trouble  can  be 
attributed  to  the  olfactory  nervous  apparatus.  It 
should  be  remembered,  too,  that  olfactory  sensations 
may  form  the  aura  of  epileptic  fits,  and  may  also  occur 
in  association  with  tumours  of  the  uncinate  convolution, 
which  is  the  cortical  centre  for  smell. 

In  hysteria  also  the  sense  of  smell  is  frequently  lost, 
usually  on  the  same  side  as  the  hemianaesthesia,  but 
occasionally  the  loss  may  be  bilateral. 

II.    THE   SECOND    OR   OPTIC   NERVE,   AND 
VISUAL   PATHS 

The  optic  nerve  contains  the  visual  fibres,  and  also 
serves  as  the  afferent  path  of  the  pupil  reflex  to  light. 

Apart  from  disease  of  the  structures  of  the  eye  itself, 
the  sight  may  be  impaired  from  lesions  which  interfere 
with  the  visual  fibres  at  any  part  of  their  course.  These 
may  be  grouped  according  as  they  are  situated  in — 

1.  The  optic  nerve. 

2.  The  optic  chiasma. 

3.  The  fibres  between  the  chiasma  and  cortex. 

4.  The  cerebral  cortex. 

Anatomical  (Fig.  16). — The  optic  nerve  runs  from 
the  eyeball  in  front  to  the  optic  commissure  behind, 
where  a  partial  decussation  of  its  fibres  with  those  of 
the  opposite  side  takes  place.  The  nerve  in  its  course 
traverses  the  orbit,  the  optic  foramen,  and  the  anterior 
fossa  of  the  skull,  and  is  invested  with  meningeal 
sheaths  continuous  with  the  dura  and  pia-arachnoid. 

The  nerve  enters  the  globe  of  the  eye  at  a  point 
slightly  below  and  on  the  inner  side  of  the  axis  of  the 
eyeball,  and,  after  piercing  the  tunics  of  the  latter, 
spreads  out  at  the  optic  disc  to  form  the  innermost  layer 
of  the  retina. 


THE   OPTIC   NERVE 


81 


The  chiasma,  or  commissure,  situated  at  the  base 
of  the  brain,  is  adherent  to  the  floor  of  the  3rd 
ventricle,  and  rests  upon  the  olivary  eminence  of 
the  sphenoid  bone ;  it  is  situated  just  anteriorly  to 
the  tuber  cinereum,  whilst  at  its  sides  the  internal 
carotid  arteries  ascend  to 
the  brain. 

At  the  commissure  a 
partial  decussation  of  the 
fibres  of  the  two  optic  nerves 
takes  place ;  thus,  the  fibres 
originating  in  the  mesial 
half  of  each  retina  cross  at 
the  chiasma  to  join  the 
optic  tract  of  the  opposite 
side,  but  those  arising  in 
the  temporal  portion  of  the 
retina  do  not  cross  the 
mesial  plane,  but  are  con- 
tinued into  the  tract  of  the 
same  side.  The  fibres  which 
are  situated  in  the  posterior 
part  of  the  chiasma  are,, 
in  all  probability,  not  con- 
nected with  vision,  but  con- 
stitute a  commissural  bundle 
running  on  each  side  into 
the  mesial  root  of  the  optic 
tract  and  connecting  the  two 
internal  geniculate  bodies 
together.  This  is  known  as 
the  commissure  of  Gudden. 

The  optic  tracts  proceed  backwards  around  the  crura 
cerebri,  and  each  divides  into  a  mesial  and  a  lateral 
root.  The  mesial  root  has  been  referred  to  above,  and 
is  probably  not  connected  with  vision ;  the  lateral  root 
ends  in  the  superior  quadrigeminal  body,  the  external 
geniculate  body,  and  the  pulvinar  of  the  optic  thalamus, 
which  structures  constitute  the  lower  visual  centres, 
Q 


Fig.  16.— Diagram  of  the  visual 
paths.    {After  D-'jerine.) 


82     DISEASES   OF   THE  NERVOUS  SYSTEM 

The  cortical  connections. — These  lower  visual  centres 
are  connected  with  the  cerebral  cortex  by  a  large  strand 
of  fibres,  known  as  the  oftic  radiation.  These  fibres  lie 
in  the  retroventricular  part  of  the  posterior  limb  of  the 
internal  capsule,  and  sweep  backwards  into  the  occipital 
lobe  on  the  outer  side  of  the  posterior  horn  of  the  lateral 
ventricle. 

Other  connections  of  the  lower  visual  centres  are — 

{a)  With  the  nuclei  of  the  ocular  nerves — probably 
this  connection  is  established  through  the  posterior 
longitudinal  bundle. 

(6)  The  superior  quadrigeminal  body  is  connected 
by  the  mesial  fillet  with  the  medulla  and  cord. 

1.  Lesions  of  the  Optic  Nerve 
optic  neuritis 

Excluding  local  conditions  in  the  eyeball  itself,  the 
causes  of  optic  neuritis  may  be  divided  into  two  classes, 
according  as  they  arise  from  {a)  intracranial  disease  or 
(6)  general  toxaemias. 

{a)  In  intracranial  disease,  meningitis,  tumours, 
and  abscesses  are  the  commonest  causes  of  optic  neuritis. 
Quite  rauely  some  faint  signs  of  inflammation  have  been 
observed  to  precede  the  atrophy  of  disseminated  sclerosis, 
tabes,  and  general  paralysis  of  the  insane.  In  meningitis 
some  degree  of  optic  neuritis  frequently  accompanies 
the  tuberculous  and  streptococcal  forms,  but  is  less  often 
met  with  in  the  cerebro-spinal  and  posterior  basic 
varieties. 

Optic  neuritis  is  the  most  constant  sign  that  accom- 
panies cerebral  tumours.  It  occurs  in  about  90  per 
cent,  of  the  cases,  and  is  of  great  diagnostic  importance. 

With  abscess  of  the  brain,  neuritis,  though  frequent, 
is  not  so  constant  as  in  tumours,  nor  when  present  is  it 
usually  so  intense. 

Ophthalmoscopic  appearances. — In  the  early  stages  the 
disc  is  hyperaemic  and  its  borders  are  ill  defined.  As 
the  process  increases,  the  physiological  cup,  of  which 
the   normal   appearance   is  shown  in  Plate  5,  Fig.  1, 


Fig.  1. — Average  normal  fundus  ; 
physiological  cup. 


Fig.  2.— Optic    neuiitis,    choked 
disc. 


Fig.  3  — Albuminuric  retinitis. 


Fig    4.  — Primary  optic  atrophy. 


PLATE  5.  —  The  fundus  oculi  in  optic  neuritis,  albuminuric 
retinitis,  and  primary  optic  atrophy,  compared  with  the  average 
normal  fundus. 


{From  Sym'.s  "  Diseases  and  Injuries  of  the  Eije.'") 


LESIONS   OF   THE   OPTIC   NERVE  83 

becomes  filled  in  and  the  course  of  the  arteries  at  its 
edges  obscured  by  inflammatory  exudate.  At  the  same 
time  the  disc  becomes  swollen  (Plate  5,  Fig.  2).  Later 
on,  changes  appear  in  the  retina.  The  veins  are  en- 
gorged and  small  capillary  flame-shaped  haemorrhages 
take  place  into  the  retinal  tissue.  Lastly,  the  exudate 
is  partially  absorbed  and  partially  organized  into 
connective  tissue  ;  the  optic  nerve  becomes  white,  the 
vessels  small,  and  permanent  failure  of  sight  ensues. 

It  is  important  to  note  that  in  the  early  stages  of 
optic  neuritis  the  patient  often  notices  little  or  no 
impairment  of  vision,  and  that  systematic  ophthal- 
moscopic and  perimetric  examination  are  the  only 
methods  which  can  be  rehed  upon  to  detect  its  presence. 

(6)  General  toxaBmias. — The  toxaemias  associated 
with  chronic  renal  disease,  severe  anaemias,  diabetes, 
chronic  lead-poisoning,  and  occasionally  other  infections, 
may  all  give  rise  to  optic  neuritis.     (Plate  5,  Fig.  3.) 

KETROBULBAR  NEURITIS 

In  retrobulbar  neuritis  there  is  an  inflammation 
followed  by  degeneration  of  the  axial  fibres  (i.e.  the 
macular  bundle).  It  is  this  condition  that  gives  rise 
to  the  toxic  amblyopias  from  tobacco  and  occasionally 
from  other  poisons. 

Symptoms. — ^Vision  is  gradually  impaired,  espe- 
cially for  colours,  so  that  the  earliest  signs  are  usually 
those  of  a  central  scotoma  for  green  and  red.  For  a 
time  there  may  be  no  changes  to  be  observed  with  the 
ophthalmoscope,  but  later  there  is  some  pallor  of  the 
disc,  especially  in  its  temporal  half. 

OPTIC   ATROPHY 

Optic  atrophy  may  occur  secondarily  to  the  con- 
ditions enumerated  under  neuritis,  or  it  may  be  due  to  a 
primary  degeneration  of  the  nerve  fibres. 

Primary  optic  atrophy  is  principally  met  with  in  tabes 
dorsalis  and  general  paralysis  of  the  insane,  and  in  dis- 
seminated sclerosis.  The  toxic  amblyopias,  as  stated 
above,  are  probably  examples  of  axial  neuritis; 


84     DISEASES   OF   THE   NERVOUS   SYSTEM 

In  primary  atrophy  the  disc  slowly  becomes  paler, 
while  the  vessels  undergo  but  little  change.  (Plate  5, 
Fig.  4.)  Vision  gradually  fails,  and  the  contraction  of 
the  colour  field  is  at  first  generally  greatest. 

HEREDITARY  OPTIC  ATROPHY    (lEBER's    DISEASE) 

The  condition — rarely  met  with — known  as  here- 
ditary optic  atrophy  was  first  described  by  Leber. 
The  disease,  which  is  characterized  by  atrophy^  of  the 
optic  nerve,  tends  to  run  in  families  and  is  usually 
transmitted  by  the  females  and  inherited  by  the  males. 
It  sometimes  skips  one  generation,  to  appear  again  in 
the  next.  In  the  majority  of  cases  the  symptoms  first 
appear  in  young  adults  (age  from  15  to  20),  but  they 
have  been  observed  to  begin  both  in  children  and  in 
elderly  people. 

The  patients  complain  of  failure  of  sight,  and  the 
ophthalmoscopic  appearances  are  those  of  partial 
atrophy,  preceded,  possibly,  by  some  slight  signs  of 
neuritis.  The  morbid  changes  occur  in  both  eyes, 
but  in  most  of  the  cases  they  do  not  progress  far  enough 
to  cause  complete  blindness.  The  symptoms  closely 
resemble  those  caused  by  tobacco,  but  in  Leber's  disease 
there  is  a  central  scotoma  for  white,  whereas  in  tobacco 
amblyopia  the  failure  of  central  vision  is  chiefly  for  red 
and  green. 

It  seems  probable  that  the  pathological  changes 
are  very  similar  to  those  of  tobacco  amblyopia,  and 
that  the  condition  is  in  reality  one  of  axial  neuritis 
(Nettleship). 

There  is  no  adequate  form  of  treatment  known. 

2.  Lesions  of  the  Chiasma 
The  chiasma  is  liable  to  be  damaged  by  tumours  at 
the  base  of  the  brain,  especially  by  those  connected  with 
the  pituitary  body  ;  hence  it  often  happens  that  patients 
with  signs  of  acromegaly  complain  first  of  their  vision. 
Reference  to  the  diagram  (Fig.  17)  shows  that  the  first 
effect  most  likely  to  be  produced  by  pressure   on  the 


LESIONS   OF  THE  CHIASMA  85 

cliiasma  is  impairment  of  the  function  of  those  fibres 
which  go  to  the  inner  side  of  each  retina  and  supply  the 
temporal  field  of  vision,  while  later  on  the  extension  of 
pressure  will  affect  the  whole  trunk  of  one  or  both  nerves. 
The  symptoms  will 
in  such  a  case  begin 
with  double  temporal 
hemianoma,  and  end  '^^^^'^^'^i- 
m  complete  atrophy. 
It  is  possible, 
though  naturally 
rare,  in  the  process 
of  atrophy  of  the 
visual  fibres  at  the 
commissure,  or  owing 
to  the  symmetrical 
position  of  two  tu- 
mours, for  the  fibres 
proceeding  to  the 
outer  half  of  each 
retina  to  be  caught, 
while  those  going  to 

the  inner  halves  are    -p.      ,_    -p..  „,„_     ,      .  ,   , , 

.  Fig.    17.— Diagram    showing   probable 

leit  intact,      in    that       effects  of  pressure  on  the  chiasma. 

case,  the  function  of 

the   outer  half   of   each   retina   being    lost,    there   will 

be  bhndness   in   both   nasal   fields,   i.e.  a  double  nasal 

hemianopia. 

3.  Lesions  between  the  Chiasma  and  the 
Cortex 

If  the  fibres  are  imphcated  anywhere  in  their  course 
between  the  commissure  and  the  cortex,  e.g.  in  the 
neighbourhood  of  the  internal  capsule,  then  the  outer 
part  of  one  half  of  the  retina  and  the  inner  part  of  the 
other  will  be  affected — ^i.e.  the  temporal  field  on  one  side 
and  the  nasal  field  on  the  other — and  thus  a  simple  or 
homonymous  hemianopia,  which  is  by  far  the  commonest 
variety,  is  produced. 


86     DISEASES  OF  THE  NERVOUS  SYSTEM 

4.  Lesions  of  the  Visual  Cerebral  Cortex 

The  area  of  cortex  concerned  with  visual  sensation 
lies  chiefly  on  the  mesial  aspect  of  each  occipital  lobe, 
above  and  below  the  calcarine  fissure. 

Destruction  of  the  visual  area  on  one  side  or  of  the 
fibres  passing  from  it  (optic  radiation)  gives  rise  to  an 
homonymous  hemianopia  of  one  or  other  side,  according 
to  the  hemisphere  affected.  From  observations  made 
during  the  War  by  Gordon  Holmes  and  Sir  W.  T.  Lister, 
it  appears  that  central  vision  is  represented  in  the 
posterior  part  of  the  occipital  areas  and  that  a  lesion  at 
the  tip  of  one  occipital  lobe  (i.e.  at  the  "  occipital  pole  ") 
disturbs  the  central  field  of  vision  in  both  eyes  by 
causing  a  small  central  scotoma  immediately  to  one  side 
of  each  fixation  point. 

The  peripheral  j^art  of  each  half  field  is  represented 
in  the  anterior  part  of  the  visual  cortex,  so  that  a  uni- 
lateral lesion  of  this  part  will  cause  some  constriction  of 
both  visual  fields  (so-called  telescopic  vision). 

In  some  cases  of  injury  to  the  visual  areas  not  only 
is  the  field  of  vision  disturbed,  but  there  is,  in  addition, 
some  loss  of  appreciation  of  depth  and  thickness  in  the 
objects  viewed. 

Wernicke's  hemiopic  pupil  reaction. — This  test  is 
carried  out  by  throwing  a  narrow  beam  of  bright  light 
on  to  the  blind  half  of  a  hemiopic  retina  and  noting  if 
the  pupil  contracts. 

Absence  of  any  contraction  is  an  indication  that  the 
lesion  causing  the  hemianopia  is  on  the  peripheral  side 
of  the  pupil  centre,  i.e.  somewhere  between  the  optic 
tract  and  the  corpora  quadrigemina ;  while  the  presence 
of  contraction  shows  that  the  lesion  is  behind  the 
centre  for  the  pupil,  i.e.  in  the  optic  radiation  or  occipital 
cortex. 

The  test  is  of  some  value  as  an  aid  in  localizing 
cerebral  tumours,  in  which  hemianopia  is  one  of  the 
symptoms,  but  it  is  not  an  easy  one  to  perform. 


LESIONS  Oi^  THE  THlKD   NERVE  87 

III.    OCULAR  NERVES 

The  3rd,  4th,  and  6th  nerves  may  conveniently  be 
considered  in  a  group,  since  they  all  supply  the  muscles 
of  the  eye. 

Lesions  of  the  Third  Nerve 

Anatomical. — The  nucleus  of  this  nerve  is  situated 
in  the  floor  of  the  aqueduct  of  Sylvius.  The  fibres 
emerge  on  the  surface  of  the  inner  side  of  the  crus  cerebri 
at  the  superior  border  of  the  pons,  and,  passing  into 
the  cavernous  sinus,  finally  enter  the  orbit  through  the 
sphenoidal  fissure  and  supply  all  the  muscles  of  the  eye- 
ball except  the  superior  oblique  (4:th)  and  the  external 
rectus  (6th).  This  nerve  also  innervates  the  levator  pal- 
pebree,  the  ciliary  muscle,  and  the  sphincter  of  the  iris. 
The  nerve  enters  the  orbit  in  two  divisions :  the  superior 
division  supplies  the  levator  palpebrse  and  superior  rectus, 
and  the  inferior  is  distributed  to  the  other  muscles. 

The  dilator  muscle  of  the  iris  obtains  its  nerve  supply 
from  the  sympathetic. 

The  whole  of  the  3rd  nerve  or  any  of  its  branches 
may  be  affected.  When  there  is  complete  paralysis 
of  the  nerve  the  upper  Hd  drops  (ptosis)  from  the  loss 
of  power  of  the  levator  palpebrse,  and  the  effort  that  is 
made  to  keep  the  eye  open  is  often  reflected  in  the 
furrowed  forehead  produced  by  the  contraction  of  the 
occipito-frontalis. 

The  eyeball  is  pulled  outwards  by  the  unantagonized 
external  rectus,  and  the  power  of  moving  it  inwards, 
upwards,  and  downwards  is  also  lost ;  all  that  can  be 
managed  is  a  slight  movement  downwards  and  inwards 
by  means  of  the  superior  oblique.  The  pupil  is  dilated 
and  unable  to  react  to  Hght  or  during  accommodation. 

When,  as  often  happens,  there  is  only  partial  para- 
lysis of  the  nerve,  one  or  more  of  the  muscles  which  it 
supplies  suffer. 

In  paralysis  of  the  superior  rectus  the  upward  and 
internal  rotatory  movements  of  the  eyeball  are  impaired. 
The  resulting  diplopia  is   crossed,  and  the  false  image 


88     DISEASES  OF  THE  NERVOUS  SYSTEM 

is  above  and  tilted  away  from  the  true  one.  When 
the  inferior  rectus  is  paralysed  the  movements  of  depres- 
sion and  external  rotation  are  impaired.  The  diplopia 
is  crossed,  and  the  false  image  is  tilted  towards  the 
true  one. 

In  paralysis  of  the  inferior  oblique  the  upward  and 
external  rotatory  movements  are  weakened.  The  diplo- 
pia is  homonymous,  the  false  image  being  above  and 
tilted  away  from  the  true  one. 

Congenital  ptosis  is  sometimes  met  with,  and  is  prob- 
ably due  to  maldevelopment  of  the  levator  palpebree. 
It  is  often  associated  with  a  deficient  power  of  moving 
the  eye  upv/ards. 

Another  class  of  cases,  also  of  congenital  origin,  is 
that  known  as  "  jaw- winkers."  In  these  there  appears 
to  be  a  deficiency  in  the  nervous  innervation  of  the 
levator  palpebrse,  while  at  the  same  time  there  is  an 
association  of  movement  between  it  and  the  muscles  of 
mastication.  The  result  is  that  of  ptosis  while  the  jaw 
muscles  are  at  rest,  and  an  opening  and  closing  of  the 
eyes  when  masticatory  movements  are  going  on. 

Lesions  of  the  Fourth  or  Trochlear  Nerve 
Anatomical. — The  nucleus  of  the  4th  nerve  is 
situated  in  the  floor  of  the  aqueduct  of  Sylvius,  just 
behind  that  of  the  3rd,  and  the  fibres  arising  from  it 
decussate  in  the  superior  medullary  velum  with  their 
fellows  of  the  opposite  side.  On  emerging  at  the  surface 
the  nerve  winds  round  the  crus  cerebri,  lying  between 
the  posterior  cerebral  and  superior  cerebellar  arteries, 
and,  after  passing  through  the  cavernous  sinus,  enters 
the  orbit  through  the  sphenoidal  fissure  to  supply  the 
superior  oblique,  which  moves  the  eyeball  downwards 
and  outwards  with  a  wheel-like  rotatory  movement 
inwards. 

Defective  movement  from  weakness  of  this  nerve  is 
not  easy  to  observe.  The  chief  symptom  is  diplopia 
with  giddiness  on  attempting  to  look  downwards,  as  in 
comino;  downstairs. 


LESIONS   OF   THE   SIXTH   NERVE         89 

The  diplopia  is  homonymous,  and  the  false  image  is 
on  a  lower  level  and  obliquely  inclined  towards  the  true 
image. 

Lesions  of  the  Sixth  Nerve,  Abducens 

Anatomical. — The  nucleus  is  in  the  floor  of  the 
4th  ventricle,  on  a  level  with  the  lower  part  of  the  pons.- 
The  fibres  emerge  from  the  groove  between  the  pons 
and  the  medulla..  Passing  through  the  cavernous  sinus, 
the  nerve  enters  the  orbit  through  the  sphenoidal  fissure, 
between  the  two  heads  of  the  external  rectus,  and 
terminates  in  this  muscle. 

In  paralysis  of  this  nerve  there  is  deficient  move- 
ment of  the  eyeball  outwards,  and  when  the  loss  of 
power  is  complete  the  eyeball  cannot  be  moved  beyond 
the  middle  line. 

There  is  internal  strabismus  owing  to  over-action 
of  the  internal  rectus,  which  in  long-standing  cases  may 
become  permanently  contracted.  There  is  diplopia  on 
attempting  to  look  outwards  on  the  paralysed  side. 
The  diplopia  is  homonymous,  and  the  true  and  false 
images  are  parallel. 

If  there  is  a  lesion  of  the  nucleus  of  the  6th  nerve 
there  will  be  paralysis  of  the  internal  rectus  of  the 
opposite  eye,  since  its  movements  are  governed  through 
fibres  from  the  nucleus  of  the  6th. 

The  general  effects  of  paralysis  of  the  ocular 
muscles  may  be  summed  up  as  follows  : 

1.  Limitation  of  movement. — When  a  muscle  is  para- 
lysed the  movement  of  the  eyeball  is  limited  in  the 
direction  in  which  the  muscle  acts.  This  can  be  shown 
by  asking  the  patient  to  follow  an  object  with  his  eyes, 
when  the  results  of  loss  of  power  in  one  or  more  directions 
will  usually  be  manifest. 

2.  Squint. — When  one  or  more  muscles  are  paralysed, 
not  only  is  there  limitation  of  movement  in  certain 
directions,  but  the  balance  of  power  when  the  eyeballs 
are  at  rest  is  also  lost,  and  the  contraction  of  the  un- 
opposed muscles  causes  the  eyeballs  to  deviate  so  that 


90     DISEASES  OF  THE  NERVOUS  SYSTEM 

their  axes  cease  to  be  parallel,  thereby  producing  the 
condition  known  as  strabismus,  or  squint. 

3.  Diplopia. — Double  vision  is  the  result  of  the 
strabismus.  The  eyeballs  having  ceased  to  be  parallel, 
the  images  no  longer  fall  on  corresponding  parts  of  the 
retinae,  and  so  two  objects  appear  to  be  present.  It  is 
shown  by  making  the  patient  follow  an  object  in  difierent 
directions,  and  then,  as  soon  as  the  affected  eye  ceases  to 
fix  that  object,  two  images  will  be  seen.  Double  vision 
does,  not,  however,  necessarily  accompany  every  case 
of  squint,  as  the  patient  sometimes  learns  to  disregard 
the  false  image. 

When  the  false  image  is  on  the  same  side  as  the 
affected  eye  the  condition  is  known  as  simple  or  homony- 
mous diplopia,  but  when  the  images  cross  so  that  the 
one  formed  on  the  retina  of  the  affected  eye  is  projected 
in  such  a  manner  that  it  is  opposite  the  sound  eye  the 
condition  is  known  as  crossed  diplopia. 

In  all  cases  of  internal  squint — convergent  strabismus 
— ^the  diplopia  is  simple.  If,  for  instance,  the  external 
rectus  of  the  left  eye  is  paralysed,  the  image  will  fall 
upon  the  retina  to  the  inside  of  the  yellow  spot,  and  in 
the  field  of  vision  it  will  appear  to  the  left  of  the  image 
belonging  to  the  right  eye. 

In  all  cases  of  external  squint — divergent  strabismus — 
the  diplopia  is  crossed.  Thus,  when  the  axes  of  the  eyes 
are  prolonged  forwards  and  the  lines  intersect  the 
diplopia  is  simple,  and  when  they  diverge  the  diplopia 
is  crossed. 

In  cases  of  paralysis  of  the  internal  and  external 
recti  the  true  and  the  false  images  will  be  parallel  to  one 
another,  but  in  paralysis  of  the  other  muscles  rotation 
of  the  eyeball  comes  into  play  and  the  false  images 
become  tilted. 

4,  Secondary  deviation  is  an  over-action  of  the  sound 
eye,  and  is  demonstrated  as  follows  :  If  the  sound  eye 
is  covered  up,  and  the  patient  made  to  "fix"  an  object 
with  the  paralysed  eye  in  the  direction  of  the  weak 
muscle,  the  eyeball  turns  as  far  as   it   can   in  the  de- 


^    LESIONS  OF  THE  SIXTH  NERVE         91 

sired  direction,  and  at  the  same  time  tlie  sound  eye 
moves  over  a  still  greater  distance  in  the  same  direction. 

If  a  man  has  a  weak  external  rectus  on  the  left 
side,  and  his  sound  eye  be  covered  up,  and  he  is  made 
to  fix  an  object  towards  his  left-hand  side  with  the 
left  eye,  the  latter  will  then  attempt  to  turn  as  far  as  it 
can  with  its  weak  external  rectus,  and  if  the  sound 
eye  is  watched  it  also  will  be  seen  to  turn  in  the  same 
direction,  but  the  balance  between  them  is  upset,  so 
that,  instead  of  the  two  remaining  parallel,  the  sound 
eye  rotates  through  a  larger  arc  than  the  weak  one. 
This  is  due  to  the  fact  that  the  left  external  rectus 
and  the  right  internal  rectus  always  act  together ; 
both  receive  the  same  amount  of  stimulus  to  look  to 
the  left,  but  on  account  of  the  weakness  of  the  external 
rectus  on  the  left  side  the  stimulus,  in  order  to  effect 
the  movement,  must  be  stronger  than  is  usually  neces- 
sary, and  the  internal  rectus  of  the  right  eye,  being 
sound,  responds  to  this  extra  stimulus,  and  so  over- 
acts and  moves  the  eyeball  over  a  distance  which  is  out 
of  proportion  to  that  of  the  left. 

5.  Erroneous  projection  of  the  field  of  vision. — This  symp- 
tom depends  on  the  limitation  of  movement  of  the 
eyeball,  so  that  objects  appear  in  one  spot  when  they 
are  in  reality  in  another.  It  is  due  to  the  fact  that  the 
relations  between  objects  and  ourselves  are  judged  by 
the  range  of  movement  of  our  eyes,  and  when  this  range 
of  movement  is  suddenly  upset  the  strong  attempt  which 
is  made  to  move  the  eye  in  the  direction  of  the  weak- 
ened muscles  occasions  the  idea  that  the  eyeball  has 
moved  farther  than  it  really  has,  and  thus  upsets  cal- 
culations of  distance  which  have  been  based  on  all 
previous  experience. 

If  the  external  rectus  on  the  left  side  be  weak,  then 
on  turning  the  eye  to  the  left  there  is  an  increased 
innervation  of  the  weak  muscle  in  order  to  try  to  pro- 
duce the  movement.  As  the  patient  has  been  in  the 
habit  of  judging  the  amount  of  movement  by  the 
amount  of  innervation  necessary  to  cause  it,  he  natur- 


92     DISEASES  OF  THE  NERVOUS  SYSTEM 

ally  interprets  this  increased  innervation  as  producing 
its  full  effect,  and  therefore  he  thinks  his  eye  has  turned 
farther  to  the  left  than  it  really  has,  and,  as  a  conse- 
quence, objects  appear  to  him  to  be  farther  to  the  left 
of  himself  than  they  really  are. 

NYSTAGMUS 

Nystagmus  is  the  term  applied  to  oscillation  of  the 
eyeballs,  which  is  usually  involuntary,  bilateral,  and 
rhythmical.  The  movements  most  commonly  occur  in 
a  horizontal  direction  (lateral  nystagmus),  and  are 
generally  manifested  only  on  turning  the  eyes  to  the 
right  or  left,  but  they  may  be  vertical  or  rotatory,  and 
in  some  instances  they  occur  while  the  eyeballs  are  at 
rest.  Nystagmus  can  be  produced  physiologically  in 
the  following  ways  : — 

1.  The  method  of  rotation  which  consists  in  turning 
a  person  round  and  round  in  a  rotatory  chair.  The 
type  of  nystagmus  (lateral,  vertical,  or  rotatory)  thus 
obtained  depends  upon  the  degree  with  which  one  semi- 
circular canal  is  stimulated  more  than  the  others,  and 
can  be  controlled  to  a  large  extent  by  the  position  in 
which  the  head  is  fixed  during  the  rotation. 

2.  The  caloric  method,  by  which  a  stream  of  hot  or 
cold  water  is  gently  run  into  the  ear. 

3.  The  passage  of  a  galvanic  current  through  the 
head. 

All  these  methods  produce  nystagmus  by  disturbing 
the  mechanism  of  the  labyrinth,  and  they  are  used  for 
testing  the  irritability  and  the  presence  of  structural 
alterations  of  this  organ. 

Nystagmus  is  the  result  of  a  disturbance  of  the 
coordinating  mechanism  for  movements  of  the  eyeball, 
and  such  disturbances  may  be  divided  into  three  groups 
according  as  they  arise  from  disorder  of  (a)  the  semi- 
circular canals  ;  (6)  the  eyes ;  (c)  the  central  nervous 
system. 

(a)  The  labyrinth,  i.e.  the  semicircular  canals,  forms 
a  peripheral  organ  from  which  impulses  pass  inwards 


NYSTAGMUS  93 

to  regulate  the  movements  of  the  eyes,  and  disease  or 
injury  of  the  canals  may  be  accompanied  by  nystag- 
mus, such  as  occurs,  for  instance,  in  some  cases  of 
acute  aural  vertigo. 

[b)  The  diseases  of  the  eye  which  set  up  nystagmus 
are  usually  those  occurring  in  childhood,  such  as  various 
kinds  of  ophthalmia  and  keratitis,  which  prevent  a 
clear  formation  of  the  image  on  the  retina.  Nystagmus 
is  also  apt  to  be  associated  with  albinism. 

(c)  In  the  central  nervous  system  nystagmus  is 
caused  by  lesions  which  interfere  with  the  paths  along 
which  coordinating  impulses  pass  between  the  semi- 
circular canals  and  the  ocular  nuclei. 

From  the  semicircular  canals  the  impulses  are  con- 
ducted along  the  vestibular  nerve  to  the  vestibular 
nucleus,  and  thence  several  communications  are  estab- 
lished, the  principal  of  which  are  those  with  the  nuclei 
of  Deiters  and  Bechterew  and  the  vermis  of  the  cere- 
bellum. It  is  probable  that  through  the  nucleus  of 
Deiters  connections  are  also  formed  with  the  dentate 
nucleus  of  the  cerebellum.  From  the  nucleus  of 
Deiters,  which  is  situated  in  the  pons,  communication 
is  established  through  the .  fibres  of  the  dorsal 
longitudinal  bundle  with  the  ocular  nuclei.  It 
thus  follows  that  lesions  which  disturb  this  delicate 
mechanism  of  coordination  are  liable  to  cause  nystag- 
mus, and  the  principal  nervous  diseases  in  which  such 
disturbance  is  apt  to  occur  are  disorders  of  the  vesti- 
bular nerve,  diseases  of  the  cerebellum  and  pons,  dis- 
seminated sclerosis,  Friedreich's  disease,  and  syringo- 
myelia of  the  medulla  and  midbrain.  Nystagmus  has 
very  occasionally  been  observed,  likewise,  in  cases  of 
alcoholic  neuritis. 

Nystagmus  is  also  associated  with  the  head- nodding 
of  children  known  as  spasmus  nutans.  Here  the  move- 
ments of  the  head,  which  often  precede  those  of  the 
eyes,  generally  begin  during  the  first  few  months  of 
life  and  tend  to  disappear  when  the  child  reaches  the 
age  of  2  or  3  years, 


94     DISEASES   OF   THE  NEKVOUS   SYSTEM 

Cases  in  which  nystagmus  appears  to  be  hereditary- 
have  been  described. 

Miner's  nystagmus. — ^Nystagmus  may  arise  in  the 
course  of  various  occupations,  and  is  so  prevalent  in 
the  case  of  miners  that  it  is  recognized  as  one  of  the 
industrial  diseases  for  the  contraction  of  which  compen- 
sation may  be  claimed. 

The  cause  of  miner's  nystagmus  has  been  attributed 
by  Snell  to  excessive  muscular  fatigue  brought  about 
by  the  position  in  which  the  miner  works,  but  recent 
investigation  seems  to  show  that  bad  illumination 
with  consequent  imperfect  formation  of  images  on 
the  retina  is  the  principal  factor,  to  which  errors  of 
refraction  may  probably  be  added  as  a  contributory 
cause  (Llewellyn). 

Symptoms  and  diagnosis  of  nystagmus. — 
When  nystagmus  has  existed  from  childhood  there  are 
often  no  symptoms,  but  when  it  arises  in  later  life 
patients  generally  complain  of  the  movements  of  objects. 

So  far  as  diseases  of  the  nervous  system  are  con- 
cerned, nystagmus  may,  generally  speaking,  be  taken 
to  indicate  the  presence  of  organic  disease,  and  it  is 
often  particularly  useful  in  aiding  the  differentiation  of 
disseminated  sclerosis  from  hysteria. 

It  must  be  distinguished  from  "  nystagmoid " 
movements,  which  are  jerks  of  the  eyeball  that  lack 
the  more  even  movement  of  nystagmus,  and  which, 
though  they  may  often  be  regarded  with  suspicion, 
have  not  in  themselves  the  same  significance  as  the 
complete  nystagmus. 

IV.    THE   FIFTH   OE  TRIGEMINAL  NERVE 

Anatomical. — This  nerve  is  partly  motor  and  partly 
sensory  in  function.  The  motor  fibres  arise  from  the 
motor  nucleus,  which  lies  in  the  grey  matter  at  the 
lateral  angle  of  the  4th  ventricle,  and  from  the  de- 
scending or  mesencephalic  root  of  the  trigeminal,  situated 
in  the  grey  matter  surrounding  the  Sylvian  aqueduct. 
The   small   motor   root   passes   forwards   beneath   the 


TEIGEMINAL  NERVE  95 

Gasserian  ganglion,  and  is  wholly  incorporated  in  the 
inferior  maxillary  division  of  the  trigeminal.  It  inner- 
vates the  temporal,  masseter,  the  external  and  internal 
pterygoid,  the  mylohyoid  and  anterior  belly  of  the 
digastric,  possibly  also  the  levator  palati  and  azygos 
uvulae  (through  Meckel's  gangUon),  and  the  tensor 
tympani  and  tensor  palati  muscles  (through  the  otic 
ganglion). 

Lesions  of  the  motor  root. — Lesions  of  this  root  are 
followed  by  loss  of  masticating  power,  and  the  lower 
jaw,  when  thrust  forwards,  is  tilted  to  the  weakened 
side  by  the  unantagonized  healthy  muscles. 

The  sensory  root  of  the  5th  nerve  ends  in  two  nuclei 
of  termination — [a)  the  sensory  nucleus  of  the  tri- 
geminal, situated  in  the  pons ;  and  (b)  the  spinal  root, 
connected  with  the  substantia  gelatinosa  of  Rolando. 

The  sensory  root  passes  with  its  motor  companion 
across  the  base  of  the  brain  and  enters  the  Gasserian 
ganglion,  which  rests  on  the  apex  of  the  petrous  portion 
of  the  temporal  bone. 

Thence  the  fibres  separate  into  three  great  divisions 
(ophthalmic,  superior  and  inferior  maxillary),  which 
supply  sensation  to  the  anterior  portion  of  the  skull, 
half  of  the  face,  including  the  orbit  and  eyeball,  nose 
and  nasal  cavity,  lips,  teeth,  mouth,  and  the  anterior 
two-thirds  of  the  tongue. 

Lesions  of  the  sensory  root. — Neuralgia  is  very  com- 
mon in  the  area  of  distribution  of  one  or  more  branches 
of  this  nerve.  Division  of  the  branches  gives  rise 
notably  to  anaesthesia,  but  often  also  to  disturbances  of 
nutrition  of  the  skin,  and  especially  of  the  eye — ^ulcera- 
tion of  the  cornea  and  conjunctiva  and  suppuration  of 
the  whole  eyeball  being  all  possibilities.  The  most 
prominent  trophic  disturbances  that  occur  over  the 
area  supplied  by  this  nerve  are,  perhaps,  those  con- 
nected with  facial  hemiatrophy. 

In  spite  of  all  these  disorders  of  nutrition  attending 
injury  of  the  nerve,  it  does  not  necessarily  follow  that 
special  trophic  fibres  exist.     Many  of  the  efiects,  especi- 


96     DISEASES   OF   THE  NERVOUS  SYSTEM 

ally  those  connected  with  the  eye,  can  be  accounted 
for  by  the  loss  of  that  protection  against  harmful  stimuli 
which  integrity  of  the  sensory  nervous  system  confers. 
The  5th  nerve  makes  many  important  connections, 
and  the  peripheral  course  of  the  fibres  subserving  the 
sense  of  taste  is  a  somewhat  complicated  one.  Two 
main  descriptions  of  the  course  of  these  taste  fibres  are 
current : 

1.  It  is  held  that  the  taste  fibres  from  the  anterior 
two-thirds  of  the  tongue  run  at  first  in  the  lingual  nerve, 
then  in  the  chorda  tympani,  to  the  geniculate  ganglion 
of  the  facial ;  subsequently  by  the  great  superficial 
petrosal  nerve  to  Meckel's  ganglion  and  the  superior 
maxillary  division  of  the  5th.  The  taste  fibres  from 
the  posterior  third  of  the  tongue  run  at  first  in  the 
glosso -pharyngeal  as  far  as  the  petrous  ganglion,  then, 
by  way  of  Jacobson's  nerve,  to  the  tympanic  plexus, 
from  which  they  follow  the  course  of  the  small  super- 
ficial petrosal  nerve  to  the  otic  ganglion,  and  thus  to 
the  inframaxillary  division  of  the  trigeminal. 

2.  Edinger  regards  the  nucleus  of  taste  as  a  mass 
of  grey  matter,  coextensive  with  the  sensory  nucleus 
of  the  5th,  which  receives  certain  afferent  fibres — (a) 
from  the  lingual  nerve,  through  the  medium  of  the 
Gasserian  ganglion ;  (6)  from  the  chorda  tympani, 
through  the  geniculate  ganglion  of  the  facial,  and  the 
pars  intermedia  ;  and  (c)  from  the  glosso-pharyngeal, 
through  the  petrous  gangUon  (Box  and  Eccles). 

Facial  Hemiatrophy 

Facial  hemiatrophy  (Fig.  18)  is  a  rare  condition, 
and  appears  to  depend  upon  a  disturbance  of  nutrition 
in  the  area  of  distribution  of  the  trigeminal  nerve.  It 
generally  first  shows  itself  during  childhood,  and  is  more 
commonly  found  in  females  than  in  males. 

Symptoms. — The  earliest  signs  are  often  found 
in  the  sldn,  which  becomes  shiny  and  pigmented  witli 
yellowish  and  brownish  patches.  The  connective  tissue 
soon  takes  part  in  the  atrophy,  and  finally  the  growth 


FACIAL   HEMIATROPHY 


97 


of  the  whole  side  of  the  face,  including  the  bones,  is  re- 
tarded. The  muscles  do  not  undergo  any  active  atrophy, 
but  they  naturally  waste  from  disuse  and  their  movements 
are  impeded  by  the  condition  of  the  tissues  around. 

The  hairs  of  the  eyebrows  often  turn  grey  or  drop 
out.  and  the  hair  of  the  head  on  the  diseased  side  is 


Fig. 


18.— Hemiatrophy  of  the  left  side  of  the  face 
and  of  the  left  half  of  the  tongue. 


sometimes  also  affected.  The  cartilage  of  the  ear  is 
usually  wasted,  and  the  corresponding  half  of  the  tongue 
is  sometimes  atrophied,  as  seen  in  the  illustration 
(Fig.  18)  ;  and  here  again  it  is  the  fat  and  connective 
tissue,  not  the  muscles,  that  disappear.  There  may  be 
deficient  sweating  on  the  affected  side ;  the  teeth  are 
often  brittle. 


98     DISEASES   OF   THE  NEKVOUS   SYSTEM 

There  is  usually  no  tendency  for  the  atrophy  to 
spread,  though  occasionally  it  extends  to  the  structures 
on  the  other  side  of  the  face.  There  is  very  seldom  any 
loss  of  sensation,  but  neuralgic  pains  may  be  felt  over 
the  wasted  area. 

Pathology. — The  area  to  which  the  atropiiy  is 
generally  confined  has  naturally  led  to  a  suspicion 
that  some  affection  of  the  5th  nerve  is  at  the  root  of 
the  trouble.  Sclerotic  changes  have  been  found  in  the 
nerve  trunk,  but  it  is  difficult  on  this  assumption  to  under- 
stand how  the  sensory  fibres  escape  so  completely. 

The  possibility  of  the  atrophy  being  due  to  changes 
in  the  sympathetic  has  been  suggested,  but  there  is  very 
little  evidence  to  support  it,  except  that  dilatation  of 
the  pupil  on  the  affected  side  has  been  noted ;  and  this 
was  well  marked  in  the  case  illustrated  in  Fig.  18. 

The  most  plausible  idea  is  that  which  regards  the 
condition  as  a  tropho-neurosis  due  to  a  failure  of  the 
trophic  function  of  the  5th  nerve  to  the  exclusion  of  its 
sensory  and  motor  functions.  This  explanation,  how- 
ever, assumes  the  existence  of  special  trophic  fibres,  of 
which  there  is  no  adequate  proof. 

Treatment. — The  wasted  parts  cannot  be  restored. 
All  that  can  be  done  is  to  maintain  the  nutrition,  general 
and  local,  at  as  high  a  level  as  possible,  and  thereby  to 
try  to  prevent  the  disease  from  extending. 

V.  THE  SEVENTH  OR  FACIAL  NERVE 
Anatomical. — This  nerve  arises  from  a  nucleus 
in  the  pons  below  the  floor  of  the  Ith  ventricle.  The 
fibres  are  at  first  in  close  relation  with  the  nucleus  of 
the  6th  nerve,  around  which  they  wind.  This  fact  is  of 
value  in  the  localization  of  cerebral  lesions,  since  their 
proximity  makes  the  two  nerves  very  liable  to  suffer 
together  from  any  gross  disease  in  the  neighbourhood 
of  their  nuclei. 

The  facial  fibres  appear  at  the  surface  of  the  brain 
at  the  angle  between  the  pons  and  cerebellum,  and,  in 
company  with  the  auditory  nerve,  but  separated  from 


PERIPHEEAL  FACIAL   PAEALYSIS         99 

it  by  the  pars  intermedia  of  Wrisberg,  they  pass  across 
the  base  of  the  brain  and  enter  the  internal  auditory 
meatus.  Here  the  facial  leaves  the  auditory  nerve  and 
enters  the  aqueductus  Fallopii,  through  which  it  passes, 
and  finally  leaves  the  skull  by  the  stylo-mastoid  fora- 
men to  supply  the  muscles  of  one  lateral  half  of  the  face 
and  the  platysma. 

During  its  passage  through  the  aqueductus  Fallopii 
the  nerve  swells  out  into  the  geniculate  ganglion,  from 
which  spring  the  greater,  lesser,  and  external  super- 
ficial petrosal  nerves,  and  it  also  gives  off,  while  passing 
through  the  canal,  a  branch  to  the  stapedius  and  the 
chorda  tympani,  which  last,  leaving  the  main  trunk  a 
little  above  the  stylo-mastoid  foramen,  turns  forwards 
and,  after  passing  through  the  tympanum  and  commu- 
nicating with  the  otic  ganglion,  joins  the  lingual  nerve, 
and  so  is  conducted  to  the  tongue  to  supply  the  anterior 
two-thirds  with  sensation  of  taste,  the  posterior  third 
being  supplied  by  fibres  from  the  glosso-pharyngeal. 

Peripheral  Facial  Paralysis  (Bell's  Palsy)  * 
Etiology. — The  principal  causes  of  peripheral  facial 
palsy  may  be  grouped  according  to  the  situation  of  the 
lesion. 

1.  In  the  pons. — The  facial  nucleus  may  be  injured 
by  tumours,  or,  more  rarely,  it  may  be  the  seat  of  an 
acute  inflammatory  process  and  form  part  of  an  acute 
poliomyelitis.  Nuclear  degenerations  are  sometimes 
met  with  in  the  course  of  cases  of  progressive  bulbar 
paralysis,  tabes,  and  disseminated  sclerosis. 

2.  At  the  base  of  the  brain. — As  the  facial  nerve 
passes  over  the  base  of  the  brain  to  reach  the  internal 
auditory  meatus  it  may  again  be  compressed  by  tumours, 
especially  by  those  which  grow  in  the  angle  between 
the  pons  and  the  cerebellum.  It  is  also  apt  to  be  in- 
jured by  gummatous  and  other  forms  of  meningitis, 
and  by  fractures  of  the  base  of  the  skull. 

*  Strictly  speaking,  "Bell's  palsy"  includes  only  that  form  in 
which,  the  lesion  occurs  after  the  nerve  has  left  the  stylo-mastoid  fora- 
men, but  the  term  is  frequently  used  for  all  forms  of  peripheral  lesions. 


100  DISEASES  OF  THE  NERVOUS  SYSTEM 

3.  In  the  bony  canal. — When  in  the  canal  the  nerve 
may  be  injured  from  disease  of  the  bone,  which  so 
frequently  arises  in  connection  with  suppuration  of  the 
middle  ear.  It  is  also  whilst  within  the  unyielding 
walls  of  the  canal  that  the  nerve  is  so  liable  to  swell  and 
be  compressed,  as  the  result  of  cold  and  toxaemias. 


Fig.  19.— Right-sided  facial  paralysislfof  peripheral  type. 
Note  the  inability  to  close  the  eye  and  the  oblitera- 
tion of  the  nasolabial  fold  on  the  paralysed  side. 

4.  Outside  the  canal. — After  the  nerve  has  emerged 
from  the  stylo-mastoid  foramen  it  may  be  injured  by 
tumours  of  the  parotid  region,  or  by  wounds  of  the  face. 

In  the  majority  of  the  cases  the  lesion  is  situated 
within  the  canal,  and  is  thought  to  be  of  a  "  rheumatic  " 
nature.  The  history  most  often  given  is  that  the  par- 
alysis came  on  after  the  patient's  exposure  to  cold,  or 


PERIPHERAL   FACIAL   PARALYSIS       101 

more  especially  a  draught  such  as  may  occur  from  the 
windows  of  a  railway  train.  It  is  generally  considered  that 
inflammation  of  the  nerve  sheath  is  the  primary  lesion, 
and  that  the  consequent  exudation,  having  no  room 
to  expand  in  the  bony  canal,  compresses  the  nerve 
fibres.  If  this  hypothesis  is  correct  these  "  rheumatic  " 
cases  may  be  regarded  as  being  analogous  to  many 
cases  of  sciatica  where  an  inflammation  of  the  nerve 
sheath  is  also  frequently  the  primary  lesion ;  but  it  is, 
of  course,  possible  that  different  toxins  give  rise  to  the 
paralysis  in  difierent  cases,  for  the  occasional  extension 
of  the  paralysis  to  the  facial  nerve  in  a  general  neuritis 
(e.g.  from  alcohol  or  diphtheria),  though  rare,  shows 
that  the  facial  nerve  is  not  immune  from  the  poisons 
which  are  more  apt  to  injure  the  nerves  of  other  parts 
of  the  body. 

Symptoms. — ^Peripheral  lesions  (i.e.  lesions  of  the 
lower  neurone  situated  at  or  below  the  nucleus)  of  the 
trunk  of  the  facial  nerve  result  in  paralysis  of  the  muscles 
supplied  by  that  nerve,  with  some  additional  symptoms 
according  to  the  locality  of  the  lesion. 

The  paralysis  being  of  the  lower-neurone  type,  the 
loss  of  power  is  accompanied  by  wasting  and  changes 
in  the  electrical  reactions.  The  paralysed  side  of  the 
face  looks  somewhat  flattened  (Fig.  19) ;  the  nasal  fold 
is  obliterated,  and  the  angle  of  the  mouth  drops,  giving 
to  the  patient  the  idea  that  it  is  "  drawn  up  "  on  the 
sound  side  ;  the  nostril  on  the  affected  side  is  somewhat 
smaller.   In  some  cases  the  paralysis  is  bilateral  (Fig.  20). 

1.  Loss  of  power. — In  a  well-marked  case  there  is 
loss  of  power  of  all  the  muscles  on  one  side  of  the  face, 
and  this  may  be  demonstrated  by  asking  the  patient 
to  perform  the  following  actions  : 

{a)  To  look  upwards. — The  forehead  will  remain 
smooth,  owing  to  paralysis  of  the  occipito-frontalis. 
(6)  To  frown. — There  will  be  no  furrow,  owing 
to  loss  of  power  in  the  corrugator  supercilii. 

(c)  To  close  the  eyes. — The  eye  on  the  affected 
side  cannot  be  closed,  and  the  attempt  to  close  it 


102  DISEASES   OF  THE  NERVOUS  SYSTEM 

is  accompanied  by  a  rolling  upwards  and  inwards 
of  the  eyeball.  This  failure  to  close  the  eye  is  due 
to  weakness  of  the  orbicularis  palpebrarum,  and 
owing  to  the  laxity  of  the  fibres  of  this  muscle  in 
the  lower  lid  the  tears  are  apt  to  run  down  the  face 
(epiphora).  Conjunctivitis  from  irritation  of  the 
unclosed  eye  may  also  arise. 

{d)  To   show  the  teeth. — The   mouth   does   not 
move  towards  the  paralysed  side. 
Other  symptoms  that  may  be  noted  are  : 

WeaJcness  of  the  buccinator  muscles,  causing  a 
tendency  for  the  food  to  stagnate  between  the 
cheek  and  the  gums. 

Affections  of  hearing. — In  some  cases  where  the 

nerves  to  the  stapedius  has  been  paralysed,  hearing 

has  been  noted  to  be  more  acute  than  usual,  due, 

it  is  thought,  to  the  unantagonized  action  of  the 

tensor  tympani  tightening  the  drum. 

2.  Loss  of  taste. — If  the  chorda  tympani  is  affected, 

taste  will  be  lost  over  the  anterior  two-thirds  of  the 

tongue  on  the  same  side  as  the  facial  paralysis. 

It  is  tested  by  placing  a  little  bitter  or  sweet  sub- 
stance (e.g.  quinine  sulphate  or  sugar)  on  the  side  of 
the  protruded  tongue,  and  asking  the  patient  to  hold  up 
his  hand  if  he  tastes  anything.  If  he  is  allowed  to  speak 
or  to  move  his  tongue  about,  the  substance  is  liable  to 
be  diffused  and  tasted  on  the  opposite  side  and  at  the 
back  of  the  tongue. 

There  is  no  loss  of  cutaneous  sensation,  but  pain 
is  sometimes  experienced  in  the  early  stage  of  the 
paralysis. 

Dia(|nosis. — There  is  usually  no  difficulty  in 
determining  whether  facial  palsy  is  present.  The  only 
condition  which  may  2)crhaps  resemble  it  superficially 
is  that  of  facial  hemiatro2)hy,  but  in  the  latter  the 
muscles,  though  rigid,  ai-e  still  capable  of  voluntary 
contraction,  and  it  is  easy  to  see  that  changes  in  the 
skin  and  connective  tissue  are  the  main  sources  of  the 
trouble. 


PERIPHERAL   FACIAL   PARALYSIS       103 

Having  decided  that  paralysis  is  present,  it  is  neces- 
sary to  consider  whether  the  cause  lies  in  the  muscles, 
the  lower  neurone  of  the  nerve,  or  the  upper  neurone. 

The  muscles  of  the  face  undergo  primary  degeneration 
in  some  of  the  myopathies,  and  weakness  is  frequently 
met  with  in  cases  of  myasthenia  gravis,  but  neither  of 


Fig.  20.— Bilateral  facial  paralysis.  Note  the 
inability  to  close  the  eyes  and  the  general 
expressionless  appearance  of  the  face. 

these  conditions  need  be  confused  with  peripheral  facial 
paralysis. 

In  the  myopathies  the  muscles  chiefly  affected  are 
those  round  the  eyes  and  at  the  angle  of  the  mouth  ; 
both  sides  are  involved,  and  in  addition  there  will  nearly 
always  be  some  signs  of  wasting  of  the  trunk  muscles. 

In  myasthenia  gravis  it  is  unlikely  that  only  one  half 
of  the  face  would  be  weakened,  and,  further,  the  varia- 
tion of  the  signs,  together  with  the  probabiUty  of  ptosis 


104    DISEASES  01^  THE  NERVOUS  SYSTEM 

and  bulbar  symptoms,  makes  it  very  unlikely  that  serious 
difficulty  would  arise  in  that  direction. 

A  primary  muscular  origin  of  the  weakness  being 
thus  excluded,  the  next  point  to  be  decided  is  whether 
the  lesion  is  in  the  lower  or  the  upper  neurone,  and  this 
it  is  usually  possible  to  do  by  noting  the  distribution  of 
the  weakness ;  for  whereas  in  lesions  of  the  lower  neurone 
there  is  generally  paralysis  of  the  whole  of  one  half  of 
the  face,  in  those  of  Ihe  upper  neurone  the  movements 
which  habitually  take  place  together  on  the  two  sides 
(bilateral  movements)  escaj^e  or  are  only  weakened. 
Thus,  whereas  in  lower- neurone  lesions  the  patient  can- 
not close  the  eye,  frown,  or  wrinkle  the  brow,  in  upper- 
neurone  lesions  all  these  actions  can  usually  be  per- 
formed. If,  however,  there  is  still  any  doubt  as  to 
which  neurone  is  affected,  it  may  usually  be  dispelled 
by  an  examination  of  the  electrical  reactions. 

Having  settled  that  the  lesion  is  situated  in  the 
lower  neurone,  one  must  next  try  to  determine  its  exact 
position. 

1.  If  the  facial  paralysis  is  accompanied  by  para- 
lysis of  the  external  rectus  on  the  same  side,  it  suggests 
(provided  there  is  no  reason  for  thinking  that  there  is 
more  than  one  focus  of  disease  present)  that  the  lesion 
is  at  the  facial  nucleus  itself,  for  it  is  there  that  the 
6th  nerve  comes  into  close  proximity  to  the  7th. 

2.  If  deafness  is  present,  it  may  be  that  both  audi- 
tory and  facial  nerves  are  injured  where  they  are  situated 
close  to  each  other  at  the  base  of  the  brain  ;  but  care 
must  be  taken  before  this  view  is  accepted,  for  it  may 
be  that  the  deafness  is  due  to  middle-ear  disease,  and 
that  the  facial  paralysis  depends  upon  necrosis  of  the 
surrounding  bone. 

3.  If  taste  is  lost,  it  is  evident  that  the  disease  is 
situated  in  that  portion  of  the  nerve  wherein  the  chorda 
tympani  runs,  i.e.  between  the  geniculate  ganglion  and 
a  point  a  little  above  the  stylo-mastoid  foramen. 

Prognosis. — The  outlook  in  a  case  of  ^^eripherai 
facial  paralysis  must,   of  course,   largely  dcjjond  upon 


PERIPHERAL    FACIAL  PARALYSIS        105 

the  cause.  Those  cases  in  which  the  neuritis  is  ap- 
parently of  a  "  rheumatic  "  nature,  and  which  appear 
to  be  caused  by  exposure  to  cold,  for  the  most  part 
do  well. 

In  those  which  follow  disease  of  the  bone  the  result 
is  more  doubtful ;  while  the  prognosis  in  those  produced 
by  new  growth  or  by  complete  severance  of  the  nerve 
must  depend  upon  the  possibility  of  removing  the  tumour 
or  of  suturing  the  nerve  trunk. 

In  the  so-called  "  rheumatic "  cases  which  so 
commonly  come  before  us,  an  incomplete  palsy  is  a 
favourable  sign  by  showing  that  the  intensity  of  the 
inflammation  is  not  great,  and  such  cases  generally 
recover  rapidly.  When  the  palsy  is  complete  the 
electrical  reactions  are  a  useful  guide.  If,  in  a  week 
or  ten  days  after  the  onset  of  the  paralysis,  reaction 
to  the  faradic  current  still  persists,  a  steady  return 
of  power  may  generally  be  predicted ;  if,  on  the 
other  hand,  the  paralysed  muscles  give  a  reaction  of 
degeneration,  recovery  will  be  much  slower  and  more 
uncertain ;  and,  lastly,  if  all  reaction  to  galvanism 
ceases,  the  outlook  is  still  worse. 

Late  results  of  facial  paralysis. — If  complete 
recovery  does  not  take  place  the  paralysed  muscles 
often  gradually  undergo  some  contraction,  which  at 
first  sight  has  the  effect  of  making  the  sound  side  appear 
to  be  paralysed,  since  the  angle  of  the  mouth  on  the 
contracted  side  is  drawn  up.  This  contraction  is  prob- 
ably accounted  for  by  changes  in  the  muscle  fibres,  and 
about  the  time  that  it  is  beginning  to  take  place  a  curious 
irritability  is  sometimes  observed,  whereby  an  attem.pt 
to  contract  one  muscle  is  accompanied  by  the  contrac- 
tion of  another  which  is  not  desired.  Eor  instance,  on 
trying  to  close  the  eye,  the  corner  of  the  mouth  on  the 
paralysed  side  may  be  simultaneously  drawn  up.  It  is 
as  though  there  were  one  sheet  of  muscle  instead  of 
individual  segments.  This  late  contraction  does  not 
appear  to  take  place  in  cases  where  the  continuity 
of  the  nerve  has  been  completely  severed.     Fibrillary 


106    DISEASES  OF  THE  NEEVOUS  SYSTEM 

tremors  may  also  take  place  in  the  paralysed 
muscles. 

Treatment. — The  best  method  of  treating  the 
"  rheumatic  "  cases  is  by  regular  faradization  of  the 
muscles  ;  their  nutrition  is  thus  maintained  so  that 
they  are  ready  to  react  to  the  impulses  as  soon  as  the 
latter  are  able  to  pass  along  the  nerve ;  possibly  the 
nutrition  of  the  nerve-endings  is  improved  at  the  same 
time.  When  there  is  no  reaction  to  the  faradic  current 
it  is  customary  to  apply  galvanism,  but  it  is  doubtful 
whether  the  latter  gives  any  better  results,  even  though 
the  muscles  react  to  it  when  they  do  not  to  faradism. 

Internally,  potassium  iodide  in  doses  of  about  5  gr. 
seems  to  be  useful ;  and,  at  the  beginning,  counter- 
irritation,  in  the  shape  of  a  blister  behind  the  ear,  some- 
times appears  to  be  of  value. 

AVhere  the  paralysis  is  due  to  necrosis  of  bone  the 
treatment  must  still  be  conducted  on  the  same  lines, 
and  in  many  cases  the  ultimate  results  are  good,  since 
the  nerve  is  generally  injured  by  extension  of  inflam- 
mation rather  than  by  pressure  of  bone  ;  and  when  the 
inflammatory  process  subsides,  power  returns,  provided 
that  the  nutrition  of  the  muscles  has  been  maintained. 

If  at  last  it  a])])ears  clear  that  the  paralysis  is  per- 
manent, the  question  of  an  operation  may  be  raised,  for 
it  has  teen  shown  to  be  jjossible  sometimes  to  restore  a 
certain  amount  of  movement  by  severing  the  facial 
nerve  and  grafting  the  end  of  the  jjcripheral  portion 
on  to  the  spinal  accessory  or  hypoglossal  nerve.  In 
most  cases,  liowcver,  the  deformity  and  inconvenience 
produced  by  the  facial  ])aralysis  are  slight,  and  are  pre- 
ferable to  the  2)roblematical  benefits  to  be  obtained 
from  an  oj)oiation  which  has  its  own  very  distinct  risks. 

Facial  Spasm 

Spasms  of  muscles,  like  paralyses,  may  be  divided 
into  three  main  groups  according  as  they  arise  from  a 
lesion  of  the  lower  neurone,  the  upper  neurone,  or  one 
of  "  functional  "  ori<rin. 


FACIAL   SPASM  107 

(a)  Of  the  lower  neiu'one  varieties  of  facial  spasm 
mention  lias  already  been  made  of  the  muscular  tremor 
which  occasionally  occurs  as  a  sequela  of  peripheral  facial 
paralysis  (p.  106).  There  is  also  the  "  tic  douloureux  " 
which  is  a  reflex  spasm  that  sometimes  accompanies 
severe  trigeminal  neuralgia,  but  w^hich  from  its  title 
must  not  be  confused  with  the  true  tics  which  are 
of  mental  origin.  Spasm  may  also  be  sometimes  pro- 
duced by  irritation  of  the  trunk  of  the  facial  nerve,  e.g. 
by  tumours,  meningeal  inflammation,  or  disease  of  bone. 

Another  form  is  a  chronic  fibrillary  tremor  which 
begins  with  a  mere  flickering  of  fibres  of  a  muscle,  often 
those  of  the  orbicularis  palpebrarum,  and  then  gradually 
spreads  over  that  half  of  the  face.  At  first  there  may 
be  long  periods  of  intermittence,  but  later,  as  the  con- 
dition progresses,  the  patient  is  hardly  ever  free.  The 
spasms  undoubtedly  vary  to  some  extent  with  the 
health  of  the  patient,  especially  in  the  earlier  stages  of 
the  trouble,  but  the  disease  is  very  intractable,  and 
drugs,  electrical  methods,  and  massage  are  all  usually 
powerless  to  produce  any  beneficial  result.  Injection 
of  alcohol  into  the  trunk  of  the  facial  nerve  has  been 
carried  out  in  some  cases  as  a  means  of  arresting  the 
spasm.  The  immediate  result  is  facial  paralysis  with 
cessation  of  tremor,  but  the  chief  drawbacks  to  this 
method  are  (1)  that  a  permanent  paralysis  may  possibly 
be  produced,  and  (2)  that  with  the  recovery  of  the 
nerve  the  tremor  is  likely  to  return.  It  is,  therefore,  a 
method  to  be  practised  only  after  careful  consideration. 

(6)  In  the  upper  neurone  any  irritation  of  cortical 
origin  may  produce  a  spasm,  and  the  author  recently 
had  such  a  case  under  observation  in  which  the  earliest 
sign  of  a  tumour  of  the  facial  area  of  the  cortex  was  a 
slight  paroxysmal  spasm  of  the  kind. 

(c)  Spasms  of  functional  nature  are  of  mental  origin 
and  come  into  the  categories  of  hysteria  and  tics,  which 
are  considered  elsewhere. 

Occasionally  chronic  facial  spasms  are  left  as  the 
result  of  chorea. 


108    DISEASES   OF   THE  NERVOUS   SYSTEM 

VI.   THE   EIGHTH   OR   AUDITORY  NERVE 

Anatomical. — The  auditory  nerve  consists  of  two 
elements — (1)  the  cochlear  nerve,  (2)  the  vestibular  nerve. 

1.  The  cochlear  nerve,  on  which  is  situated  the 
ganglion  spirale,  divides  directly  into  branches  to  (1) 
the  macula  acoustica  of  the  saccule,  (2)  the  ampulla 
of  the  posterior  semicircular  canal,  (3)  the  organ  of 
Corti  in  the  cochlea.  Centrally,  the  fine  fibres  of  the 
cochlear  nerve  end  in  a  ganglion  in  intimate  relation 
with  the  restiform  body,  which  may  be  described  as 
consisting  of  two  parts  :  (a)  tuberculum  acousticum,  or 
lateral  cochlear  nucleus ;   {b)  ventral  cochlear  nucleus. 

Central  connections  of  the  cochlear  nerve. — From  the 
cells  of  these  nuclei  two  tracts  arise  :  a  ventral  tract, 
composed  of  the  fibres  of  the  corpus  trapezoides,  and  a 
dorsal  tract,  represented  by  the  strise  acousticae.  These 
tracts  cross  the  middle  line,  decussating  with  the  corre- 
sponding fibres  of  the  opposite  side,  and  then  the  strand 
turns  upwards  to  become  the  lateral  fillet ;  the  fibres 
of  the  lateral  fillet  end  in  the  inferior  quadrigeminal 
body  and  the  corpus  geniculatum  internum,  and  from 
the  latter  a  tract  proceeds  to  the  cortex  of  the  superior 
convolution  of  the  temporal  lobe.  But  it  should  be 
noted  that  certain  internodes  are  situated  in  the  corpus 
trapezoides  and  the  lateral  fillet,  of  which  the  most 
important  is  the  superior  olive,  by  means  of  which  a 
communication  is  established  with  the  nuclei  of  the 
3rd,  4th,  and  6th  nerves.  In  this  way  the  organ  of 
hearing  is  brought  into  connection  with  the  nuclei  which 
preside  over  the  movements  of  the  eyeballs. 

The  succession  of  neurones  which  build  up  the  sensory 
chain  linking  the  organ  of  hearing  to  the  cortex  are 
therefore — 

(1)  Bipolar  cells  of  the  ganglion  spirale. 

(2)  Neurones  of  the  terminal  cochlear  nuclei; 

(3)  Neurones  of  the  superior  olive  and  nucleus  of 

lateral  fillet. 

(4)  Neurones  of  the  corpus  geniculatum  internum. 


AUDITORY   NERVE    LESIONB  109 

2.  The  vestibular  nerve,  on  which  is  the  vestibular 
ganglion,  enters  the  brain  on  the  mesial  side  of  the 
ventral  cochlear  nucleus  ;  its  fibres  end  in  three  nuclei 
of  termination  :  {a)  principal  dorsal  nucleus,  (b)  nucleus 
of  Deiters,  and  [c)  perhaps  "  nucleus  of  descending 
root." 

Central  connections  of  the  vestibular  nerve. — Their 
connection  with  the  nucleus  of  Deiters  brings  the  fibres 
of  the  vestibular  nerve  into  relationship  with  other 
parts  of  the  mechanism  of  equilibration,  for  Deiters' 
nucleus  constitutes  an  internode  in  the  path  of  those 
fibres  which  connect  the  cortex  and  roof  nuclei  of  the 
cerebellum  with  the  motor  apparatus  of  the  spinal 
cord  ;  it  also  receives  fibres  through  the  vestibular  nerve 
from  the  labyrinth  of  the  ear,  and  sends  fibres  into  the 
posterior  longitudinal  bundle,  through  which,  in  all 
probability,  it  influences  the  nuclei  of  the  ocular  nerves. 

Lesions  oi  the  auditory  nerve. — The  chief  symp- 
toms of  disorder  of  the  auditory  nerve  are  deafness, 
tinnitus,  and  vertigo  (Chapter  LIV.).  The  nerve  is  very 
liable  to  injury,  often  in  conjunction  with  the  facial, 
from  the  pressure  of  tumours,  especially  those  growing 
at  the  cerebello-pontine  angle,  and  it  may  also  be 
damaged  along  with  the  facial  in  fractures  of  the  base 
of  the  skull. 

Atrophy  of  the  auditory  nerve  sometimes  occurs  in 
locomotor  ataxy. 

VII.  THE   GLOSSO-PHARYNGEAL  AND  VAGUS 

NERVES 

Anatomical. — The  motor  or  efferent  fibres  spring 
from  the  dorsal  motor  nucleus  and  the  nucleus  ambiguus, 
lying  deeply  in  the  substance  of  the  medulla  ;  these 
fibres  join  the  vagus  and  glosso-pharyngeal  nerves. 

The  afferent  fibres  of  the  glosso-pharyngeal  and 
vagus  end  in  the  dorsal  nucleus  and  in  the  funiculus 
solitarius. 

The  glosso-pharyngeal  or  9th  nerve  emerges  from 
the  upper  part  of  the  medulla  oblongata,  and  leaves 


no    DISEASES  OF  THE  NERVOUS  SYSTEM 

the  cranium  by  the  jugular  foramen.  It  then  appears 
in  the  interval  between  the  internal  jugular  vein  and 
the  internal  carotid  artery,  and  is  directed  downwards 
and  forwards  to  the  tongue.  It  distributes  branches  to 
the  mucous  membrane  of  the  tongue,  pharynx,  and 
middle  ear,  as  well  as  to  the  stylo-pharyngeus  and 
possibly  to  the  middle  constrictor  of  the  pharynx. 

The  vagus  or  10th  nerve,  which  also  leaves  the  skull 
by  the  jugular  foramen,  is  directed  downwards  in  the 
carotid  sheath,  and  enters  the  thorax  and  abdomen. 
Each  vagus  is  connected  with  the  following  cranial 
nerves  :  spinal  accessory,  glosso-pharyngeal,  facial,  and 
hypoglossal ;  also  with  some  spinal  nerves,  and  with 
the  sympathetic  in  the  neck,  thorax,  and  abdomen. 

Lesions  of  the  glosso-pharyngeal. — The  exact 
functions  of  this  nerve  are  not  known,  owing  to  the 
rarity  of  cases  of  isolated  paralysis,  since  it  is  intimately 
connected  with  the  fibres  of  the  vagus.  Sense  of  taste 
may  be  impaired  in  the  posterior  third  of  the  tongue, 
and  there  is  some  difficulty  in  swallowing,  owing  prob- 
ably to  paralysis  of  the  stylo-pharyngeus. 

Lesions  of  the  vagus. — Affections  of  the  nuclei 
in  the  medulla  are  accompanied  by  unilateral  or  bilateral 
palsies.  Lesions  of  one  nerve  cause  unilateral  paralysis 
of  the  soft  palate  and  larynx  (probably  through 
spinal  accessory  fibres),  as  is  shown  by  the  uvula  being 
drawn  over  to  the  healthy  side  on  phonation  and  by 
one  vocal  cord  assuming  a  position  nearly  midway  be- 
tween abduction  and  adduction — the  so-called  "  cada- 
veric ])osition."  The  paralysed  cord  does  not  move 
either  during  phonation  or  respiration,  but,  on  phonation, 
the  healthy  cord  moves  over  the  middle  line  to  try  to 
meet  it.  Owing  to  their  length,  one  or  other  vagus  nerve 
is  particularly  likely  to  be  damaged,  and  the  left  recur- 
rent laryngeal,  which  winds  round  the  arch  of  the  aorta, 
is  especially  ai)t  to  suffer  from  pressure  of  aneurysms. 
In  this  case  the  paralysed  cord  assumes  the  position 
described  above  ;  the  voice  is  hoarse  and  weaker  than 
usual,  the  cough  is  harsh,  and  the  power  of  coughing  is 


LESIONS    OF   THE    VAGUS  111 

mucli  diminished  owing  to  the  inabiUty  to  bring  the 
cords  firmly  together. 

The  fibres  to  the  abductors  of  the  vocal  cords  are 
more  easily  damaged  than  those  to  the  adductors  ;  hence 
in  the  earlier  stages,  before  the  paralysis  is  complete, 
the  unantagonized  adductors  are  apt  to  draw  the  cord 
to  the  middle  line  (Semon)  till  they,  in  their  turn,  lose 
their  function,  and  the  cord  swings  into  its  position  of 
complete  paralysis.  During  the  stage  in  which  one 
abductor  only  is  affected  there  are  no  definite  symptoms 
to  draw  the  patient's  attention  to  his  throat ;  the  move- 
ments of  the  healthy  cord  are  sufiicient  for  ordinary 
respiration,  and,  since  the  two  cords  can  meet  in  the 
middle  line,  vocalization  is  not  seriously  interfered  with. 
This  stage  of  the  paralysis  is  therefore  likely  to  remain 
undiscovered  unless  a  systematic  examination  of  the 
larynx  is  made. 

Both  cords  may  be  paralysed  if  the  nuclei  of  the 
nerves  are  diseased,  in  which  case  the  voice  and  the 
power  to  cough  are  both  completely  lost. 

In  paralysis  of  both  abductors  the  cords  come  to- 
gether in  the  middle  line,  and  they  do  not  separate 
during  inspiration.  The  voice  and  power  of  coughing 
are  but  little  afiected,  but  there  is  obstruction  to 
respiration,  which  shows  itself  by  loud  stridor. 

If  the  abductor  is  paralysed  on  one  side  only,  the 
cord  on  that  side  will  approach  the  middle  line,  but 
there  will  be  no  definite  symptoms  so  long  as  the  move- 
ments of  the  other  cord  are  freely  carried  out.  This, 
as  already  mentioned,  is  apt  to  occur  from  pressure 
on  the  recurrent  laryngeal  before  the  adductors  are 
paralysed. 

In  adductor  paralysis  the  cords  cannot  be  brought 
together,  and  the  voice  is  lost.  This  form  of  paralysis 
is  usually  only  partial,  and  is  the  form  which  commonly 
occurs  in  hysteria,  when  the  cords  cannot  be  voluntarily 
brought  together  for  phonation,  but  usually  move  freely 
for  coughing,  and  therefore  the  patient  may  be  able 
to  cough  although  the  voice  is  lost. 


112    DISEASES   OF   THE  NERVOUS   SYSTEM 

Spasm  of  the  muscles  of  the  larynx  may  occur  from 
central  or  peripheral  irritation. 

The  result  of  general  laryngeal  spasm  is  closure  of 
the  glottis  owing  to  the  predominating  power  of  the 
adductors.  One  of  the  commonest  forms  of  laryngeal 
spasm  occurs  in  rickety  children,  under  the  name 
of  laryngismus  stridulus.  The  attacks,  which  occur 
chiefly  at  night,  are  characterized  by  acute  dyspnoea, 
which  lasts  for  some  seconds,  followed  by  loud  crow- 
ing inspirations  as  relaxation  of  the  muscles  takes 
place. 

Laryngeal  spasm  occurs  also  in  epilepsy  and  occa- 
sionally in  hysteria.  It  may  be  induced,  too,  by  local 
irritation,  such  as  from  foreign  bodies  or  from  tuber- 
culous or  syphilitic  ulceration  of  the  larynx. 

The  respiratory  and  cardiac  rhythms  may  also  be 
modified  from  disease  of  the  vagus. 

VIII.  THE  ELEVENTH  OR  SPINAL  ACCESSORY 

NERVE 

Anatomical. — The  spinal  accessory  nerve  con- 
sists of  two  divisions.  The  spinal  division  arises  by  a 
series  of  filaments  from  the  cells  of  the  anterior  horns 
from  the  1st  to  the  5th  cervical  segments,  and  passes 
upwards  through  the  foramen  magnum  to  join  the 
accessory  portion,  in  company  with  which  it  leaves 
the  skull  by  the  jugular  foramen,  and,  after  passing 
either  in  front  of  or  behind  the  internal  jugular  vein 
and  perforating  the  sterno-mastoid,  crosses  the  pos- 
terior  triangle  of  the  neck,  to  end  in  the  trapezius. 
It  supplies  this  latter  muscle  as  w^ell  as  the  sterno- 
mastoid. 

The  accessory  division  is  to  be  regarded  rather  as  an 
extension  of  the  vagus.  It  arises  from  nuclei  in  con- 
tinuation with  those  of  the  vagus,  and,  after  passing  out 
of  the  skull  with  the  spinal  portion,  leaves  it  to  return 
again  to  the  vagus ;  and  it  ultimately  supplies  fibres  to 
the  pharyngeal  and  superior  and  recurrent  laryngeal 
nerves. 


THE    SPINAL   ACCESSORY   NERVE        113 

The  spinal  accessory,  like  the  vagus,  is  apt  to  be 
compressed  by  tumours  and  to  have  its  nuclei  affected 
in  bulbar  lesions. 

The  results  of  paralysis  of  the  spinal  portion  are  loss 
of  power  and  wasting  of  the  sterno-mastoid  and  upper 
part  of  the  trapezius. 

Paralysis  of  one  sterno-mastoid  gives  rise  to  dij[!i- 
culty  in  rotating  the  head  to  the  opposite  side,  and 
when  power  is  lost  in  both  there  is  a  tendency  for  the 
head  to  fall  back. 

When  the  upper  and  middle  fibres  of  the  trapezius 
are  paralysed  the  movements  of  shrugging  the  shoulders 
and  raising  the  arm  above  the  horizontal  position  are 
impaired,  but  the  latter  movement,  though  weakened, 
can  still  be  accomplished  by  means  of  the  serratus 
magnus. 

When  the  lower  fibres  are  paralysed  the  position  of 
the  scapula  is  altered,  so  that  the  vertebral  border  stands 
out  prominently,  as  in  paralysis  of  the  serratus  magnus, 
and  there  is  also  some  loss  of  power  to  rotate  the  arm 
backwards. 

The  sterno-mastoid  and  trapezius  often  suffer  to- 
gether, but  the  nerve  is  sometimes  affected  after  it  has 
left  the  sterno-mastoid,  in  which  case  the  trapezius 
suffers  alone. 

The  sterno-mastoid  and  trapezius  receive  fibres  also 
from  the  2nd  and  from  the  3rd  and  4th  cervical  nerves 
respectively. 

IX.  THE  TWELFTH  OR  HYPOGLOSSAL  NERVE 

Anatomical. — This  nerve  arises  from  a  nucleus 
situated  in  the  floor  of  the  4th  ventricle,  and  its  fila- 
ments appear  on  the  anterior  surface  of  the  medulla 
near  the  middle  line,  whence,  uniting  into  a  trunk,  they 
pass  through  the  anterior  condyloid  foramen. 

The  muscles  chiefly  affected  in  lesions  of  the 
hypoglossal  are  those  of  the  tongue. 

Lesions  of  the  nucleus  of  the  flbres  of  the  nerve 
cause  loss  of  power,  perhaps  «t  first  only  showing  itself 

I 


lU    DISEASES   OF  THE   NERVOUS   SYSTEM 

by  indistinct  pronunciation  of  certain  letters,  and  later 
by  paralysis  so  that  the  tongue  cannot  be  put  out  or 
thrust  into  either  cheek.  As  the  muscle  atrophies  the 
mucous  membrane  becomes  thrown  into  folds  (Eig.  21 ). 

When  the  muscles  of  one  half  of  the  organ  are  weak- 
ened the  tongue,  if  protruded,  will  deviate  towards,  the 
weakened  side. 

The  nerve  supply  of  the  orbicularis  oris,  though 
running  in  the  trunk  of  the  facial,  appears  to  come  from 


Fig.  21.— Atrophy  of  the  tongue  niiist-les. 
Note  the  wriukled  surface. 


the  hypoglossal  nucleus,  and  hence  paralysis  of  the  lips 
of  nuclear  origin  is  liable  to  accompany  that  of  the 
tongue,  as,  for  instance,  often  happens  during  the 
course  of  progressive  bulbar  paralysis. 

The  hypoglossal  nucleus  is  especially  liable  to  be  the 
seat  of  degeneration  in  progressive  bulbar  paralysis  ; 
it  may  also  be  affected  in  syringomyelia  of  the  medullary 
region  and  by  the  pressure  of  tumours. 

At  the  base  of  the  skull,  meningitis  and  tumours 
may  compress  the  nerve  trunks. 


CHAPTER    XII 

PARALYSES    OF   THE    UPPER   LIMB 

Anatomical. — The  brachial  plexus  is  made  up  of  the 
5th,  6th,  7th,  and  8th  cervical  roots,  together  with 
the  1st  dorsal  root.  The  fibres  of  the  roots  enter 
into  the  composition  of  different  nerve  trunks,  so 
that  the  distribution  of  the  paralysis  from  a  root 
lesion  differs  from  that  due  to  a  lesion  of  a  peripheral 
nerve. 

It  is  thus  necessary,  for  purposes  of  localization,  to 
distinguish  between  paralyses  of  root  ^distribution  and 
those  of  peripheral  nerve  origin. 

On  the  next  page  will  be  found  the  table  compiled 
by  Wilfred  Harris*  as  the  result  of  stimulation  of  nerves 
in  operation  cases,  of  observations  on  pathological 
conditions,  and  of  dissections  of  the  plexus.  Harris 
points  out  that  certain  variations  may  be  met  with 
through  plexuses  being  shifted  either  up  or  down.  If 
the  plexus  is  a  high  one  (i.e.  prefixed)  it  receives  a 
branch  from  the  4th  cervical  nerve  with  some  curtail- 
ment of  the  branches  from  the  lowest  roots.  If,  on 
the  other  hand,  the  plexus  is  a  low  one  (i.e.  postfixed) 
there  will  be  no  branch  from  the  4th  cervical  nerve, 
and  the  roots  at  the  lower  end  will  be  of  large  size. 
These  differences  entail  slight  variations  in  the  distri- 
butions of  the  roots.  The  table  is  from  a  prefixed 
type  of  plexus. 

*  "The  True  Form  of   the  Brachial  Plexus  and  its  Motor  Dis- 
tribution," Journ.  of  An  at.  and  Phys.,  July,  1904. 

116 


116    DISEASES   OF  THE  NERVOUS  SYSTEM 

Motor  Root  Supply  of  Muscles  of  Upper  Extremity 

{Wilfred  Harris) 
PREFIXED   TYPE   OF   PLEXUS 

NERVE    ROOTS  MUSCLES 

4-5th  C.       .     .     Deltoid. 

Teres  minor. 

Supraspinatus. 

Infraspinatus. 

5th  C.     .     .     .     Rhomboids. 

J Subclavius. 

Biceps. 

Brachialis  anticus. 
Supinator  longus. 
Supinator  brevis. 
Pronator  radii  teres. 
"Extensor  carpi  radialis  longior. 
Extensor  carpi  radialis  brevier. 

'5-6th  C.       .     .     Clavicular  pectoral. 
Subscapularis. 

5-6-7th  C.  .     .     Triceps. 

Serratus  magnus. 

G-7th  C.      ,     .     Teres  major. 

Coraco -brachial!  s 

6-7- 8th  C.  .     .     Pectoralis  major  (sternal). 
Latissimus. 

Extensor  carpi  ulnaris. 
Flexor  carpi  radialis. 

7-8th  C.      .     .     Flexor  carpi  ulnaris. 
Pectoralis  minor. 
Extensor  ossis  metacarpi  pollicis. 
Extensor  primi  internodii  pollicis. 
Extensor  communis  digitorum. 

8th  C.-lst  D.   .     Extensor  secundi  internodii  pollicis. 
Extensor  indicis. 
Extensor  minimi  digiti. 
Pronator  quadratus. 
Palmuris  longus. 
Flexor  sublimis  digitorum. 
Flexor  longus  pollicis. 
Flexor  profundis  digitorum. 

1st  D.     .     .     .     Thenar  muscles. 
Lumbricals. 
[Interossei. 
llypothenar  muscles. 


BRACHIAL   PLEXUS    PARALYSES         117 

It  is  occasionally  possible,  in  cases  of  injury  of  the 
cord,  to  analyse  further  the  relative  positions  of  the 
segments  from  which  the  muscles  are  innervated.  I 
have  recorded  cases*  from  which  it  appears  that  the 
extensors  and  flexors  of  the  wrist  are  situated  at  a  higher 
level  of  the  cord  than  the  long  flexors  and  extensors  of 
the  fingers  and  thumb,  and,  further,  that  the  extensors 
of  the  wrist  are  represented  at  a  higher  level  in  the  cord 
than  the  flexors  of  the  wrist. 

The  motor  and,  of  course,  in  many  cases  the  sensory 
roots  are  especially  liable  to  suffer  in  injuries  of  the 
spinal  column,  e.g.  fractures  and  dislocations,  and 
Farquhar  Buzzard  has  shown  that  uniradicular  palsies 
apparently  depending  upon  a  vascular  lesion  may 
sometimes  occur.  The  whole  or  part  of  the  brachial 
plexus  may  be  severely  injured  by  dislocations  of  the 
head  of  the  humerus,  or  in  elderly  people  it  may  be 
subject  to  inflammations  which  appear  to  be  of  a  rheu- 
matic or  gouty  nature. 

The  peripheral  nerves  may  be  crushed  or  severed, 
they  may  suffer  as  part  of  a  more  generalized  neuritis, 
or  they  may  be  injured  by  continual  pressure  as  from 
the  use  of  crutches. 

Paralyses  of  the  Brachial  Plexus 
For  purposes  of  classification,  the  paralyses  of  the 
brachial   plexus    may   be    grouped   in   three    divisions, 
though,  of  course,  they  are  often  mixed : 

1.  Paralyses  of  the  upper  type,  in  which  the  loss 
of  power  corresponds  to  the  distribution  of  the  5th 
cervical  root. 

2.  Paralyses  of  the  lower  type,  in  which  the 
weakness  chiefly  follows  the  supply  of  the  1st 
dorsal  root. 

3.  Paralyses  of  the  intermediate  type,  in  which 
the  fault  lies  in  the  6th,  7th,  and  8th  cervical  roots. 

The  5th  cervical  root,  owing  to  its  anatomical  posi- 
tion, is  the  one  likely  to  sufier  most  when  the  neck  and 
*  Brain,  Part  Isxxv.,  1899. 


118    DISEASES   OF  THE  NERVOUS   SYSTEM 

shoulder  are  forcibly  stretched  apart.  This  sometimes 
happens  to  a  child  as  it  is  being  born,  with  the  result 
that  the  deltoid,  biceps,  and  supinator  longus  are  para- 
lysed, and  occasionally  other  roots  of  the  plexus  are 
also  affected.  The  condition  is  often  known  as  obste- 
trical paralysis.  The  arm  is  usually  kept  extended 
and  rotated  inwards  by  the  unantagonized  action  of 
the  triceps  and  other  muscles.  "Whether  recovery  takes 
place  or  not  depends  upon  the  degree  of  primary  injury, 
though  happily  in  many  cases  the  power  of  conduc- 
tivity returns. 

The  Hne  of  treatment  consists  in  keeping  the  para- 
lysed muscles  relaxed  and  preserving  as  far  as  possible 
their  nutrition  by  means  of  massage  and  electricity,  until 
the  functions  of  the  nerve  fibres  are  restored.  If  after 
some  months  there  be  no  sign  of  any  return  of  power, 
and  no  encouragement  be  obtained  from  the  electrical 
reactions,  the  chances  that  the  injured  nerve  will  re- 
cover are  very  slight.  In  cases  which  show  no  signs 
of  recovering,  the  possibility  of  obtaining  any  im- 
provement from  an  operative  procedure  by  graft- 
ing the  injured  nerve  on  to  a  healthy  one  must  be 
considered. 

The  8th  cervical  and  1st  dorsal  roots  are  sometimes 
injured,  as  they  leave  the  cord,  by  accessory  ribs,  tumours, 
or  thickening  of  the  meninges  (Plate  6).  This  variety 
is  known  as  lower  plexus  or  Klumpke's  paralysis. 
The  small  muscles  of  the  hand  waste,  and  there  is 
some  pain  or  anaesthesia  over  the  area  supplied  by 
the  roots.  In  some  cases  the  oculo-pupillary  fibres  arc 
implicated,  with  consequent  miosis  and  narrowing  of 
the  palpebral  fissure.  The  segments  of  the  cord  sup- 
plying the  muscles  of  the  hand  are  especially  apt 
to  be  affected  in  progressive  muscular  atrophy  and 
syringomyelia. 

Cervical  rib. — The  symptoms  of  a  cervical  rib 
arc  produced  by  pressure  on  the  roots  of  the  brachial 
plexus,  the  1st  dorsal  root  being  the  one  most  commonly 
affected. 


CEEVICAL   RIB  ll9 

Symptoms. — The  patient  usually  complains  of  weak- 
ness of  the  hand,  together  with  pain,  tingling,  and 
numbness  chiefly  along  the  ulnar  border  of  the  fore- 
arm, and  on  examination  some  anaesthesia  can  often  be 
demonstrated  over  this  part,  i.e.  over  the  area  supplied 
by  the  1st  dorsal  root.  The  weakness  is  accompanied 
by  wasting  of  the  small  muscles  of  the  hand,  and  Kinnier 
Wilson  has  pointed  out  that  when  the  muscles  of  the 
thumb  are  affected  the  wasting  is  often  limited  to  the 
abductor  and  opponens  pollicis,  a  distribution  which 
is  in  contrast  with  that  found  in  progressive  muscular 
atrophy,  where  all  the  thumb  muscles  suffer  equally, 
and  which  may  thus  be  an  additional  point  of  diag- 
nostic importance  in  differentiating  between  the  two 
diseases.  Signs  of  pressure  on  the  cervical  sympathetic 
fibres  may  be  met  with,  as  mentioned  in  the  preceding 
paragraph. 

The  symptoms  caused  by  cervical  ribs  usually  come 
on  comparatively  late  in  life,  and  the  cause  of  this  late- 
ness of  onset  has  given  rise  to  considerable  speculation. 
It  has  been  attributed  to  late  ossification  of  the  cervical 
rib,  but,  as  pointed  out  by  Wood  Jones,  (1)  there  is  no 
evidence  that  a  cervical  rib  does  ossify  late,  and  (2) 
this  hypothesis  must  fail  when  a  normal  first  rib  is  the 
offender.  The  explanation  suggested  by  this  observer 
is  that  the  symptoms  are  brought  about  by  a  change  in 
the  relative  positions  of  the  different  parts  of  the  shoulder 
girdle  consequent  on  general  loss  of  muscular  tone.  It 
is  thought  that  the  dropping  of  the  outer  end  of  the 
clavicle  which  is  apt  to  occur  in  such  circumstances 
may  lead  to  tension  arising  between  the  lowest  cord  of 
the  plexus  and  the  cervical  rib ;  and  in  support  of  this 
view  it  has  been  shown  that  symptoms  can  often  be 
relieved  by  voluntary  elevation  of  the  shoulder  girdle. 
In  some  cases  the  symptoms  appear  to  be  caused  by  a 
ligamentous  structure  attached  to  the  rib  rather  than 
by  the.  rib  itself. 

Diagnosis. — The  diagnosis  is  not  always  easy,  and  it 
must  not  be  too  hastily  concluded  that  the  presence  of 


120    DISEASES   OF   THE   NERVOUS   SYSTEM 

a  cervical  rib — which,  as  shown  by  the  X-rays,  is  not 
an  uncommon  occurrence — is  necessarily  the  cause  of 
the  symptoms. 

Where  sensory  disturbance  alone  is  complained  of, 
the  condition  must  be  differentiated  from  those  symp- 
toms of  tingling,  pain,  and  numbness  often  known  as 
acroparcBstJiesia,  which  are  frequently  complained  of  by 
women  and  appear  to  depend  upon  vaso -motor  derange- 
ment quite  independently  of  any  gross  anatomical 
lesion. 

Assistance  in  arriving  at  a  conclusion  will  be  derived 
from  observation  of  the  distribution  of  the  symptoms. 
When  a  cervical  rib  is  the  cause  the  sensory  disturbance 
will  tend  to  be  distributed  over  a  root  area,  usually  that 
of  the  1st  dorsal ;  while  in  acroparsesthesia  of  general 
origin  the  symptoms  are  more  widely  distributed  and 
often  occur  in  both  arms  and  possibly  in  the  legs  as  well. 
When  wasting  of  the  small  muscles  of  one  hand  is  the 
prominent  symptom  the  possibility  of  progressive  mus- 
cular atrophy  or  syringomyelia  has  to  be  thought  of, 
and  here  the  escape  of  certain  small  muscles  of  the 
thumb  in  the  cervical  rib  cases,  and  the  distribution 
and  nature  of  the  sensory  changes,  are  points  that  will 
be  of  assistance. 

The  most  conclusive  cases  are  those  in  which  wasting 
and  sensory  changes  of  gradual  onset  are  associated 
with  the  presence  of  a  cervical  rib  and  are  confined  to 
the  distribution  of  the  1st  dorsal  root. 

Lesions  o£  iieriphcral  nerves. — Lesions  of  nerves 
below  the  level  of  the  plexus  give  rise  to  symptoms 
corresponding  to  the  peripheral  nerve  distribution.  The 
accurate  investigation  of  these  is  of  the  greatest  im- 
portance in  view  of  the  surgical  procedure  which  is  so 
often  required.  The  signs  of  injury  to  a  peripheral 
nerve  may  be  summarized  briefly  as  follows  : — 

1.  Movement. — The  motor  effects  of  a  peripheral 
nerve  injury  are  those  already  described  under  the 
heading  of  Lower  Motor  Neurone  Lesions,  and  can  be 
recognized  by  (i.)  loss  of  jjower,  (ii.)  muscular  wasting, 


LESIONS   OF    PERIPHEEAL    NERVES     121 

(iii.)  loss  of  reflexes  in  the  affected  area,  (iv.)  clianges  in 
the  electrical  reactions,  (v.)  late  deformities. 

2.  Sensation. — The  three  peripheral  sensory  systems 
for  (a)  light  touch,  (b)  pain,  and  (c)  deep  sensibility,  as 
described  at  p.  12,  must  all  be  carefully  tested,  and  it 
must  be  remembered  that  the  areas  over  which  these 
are  affected  by  no  means  always  correspond  with 
one  another.  Thus,  when  a  peripheral  nerve  such  as 
the  ulnar  is  severed,  there  will  be  no  loss  of  deep  sensi- 
bility unless  the  tendons  along  which  the  nerves  for 
deep  sensibility  run  are  also  severed,  and  so  it  is  possible 
for  the  patient  to  recognize  pressure  sensations  when  he 
cannot  feel  those  of  light  touch.  Moreover,  Head  has 
shown  that  the  proportionate  effects  on  the  different 
peripheral  sensory  systems  vary  according  as  to  whether 
the  lesion  is  nearer  the  periphery  or  the  cord. 

The  more  distal  the  peripheral  lesion — i.e.  the  nearer 
it  is  to  the  hand  or  foot,  as  in  the  case  of  division  of  the 
median  or  ulnar  nerves — the  greater  will  be  the  extent 
of  the  loss  to  light  touch  as  compared  with  that  of  pin- 
prick. 

As  the  position  of  the  lesion  gets  nearer  the  central 
nervous  system,  as  in  lesions  of  the  brachial  plexus, 
the  loss  of  the  sensation  to  pin-prick  increases  in  area 
until  it  coincides  with  that  of  touch,  while  on  the  central 
side  of  the  plexus,  as  in  a  lesion  of  the  posterior  roots, 
the  area  of  loss  to  pin-prick  may  actually  exceed  the 
extent  of  that  for  light  touch.  The  proportionate  area 
between  loss  of  pin-prick  and  of  Hght  touch  may  thus 
give  some  indication  to  the  position  of  the  lesion. 

3.  Vaso-motor  and  trojohic  symjJtoms. — Dryness,  cya- 
nosis, and  coldness  of  the  skin,  which  also  easily  ulcerates 
after  any  slight  injury,  are  all  common  results  of  peri- 
pheral nerve  injuries.  The  injured  part  may  become 
oedematous  if  kept  in  a  dependent  position,  and  all  the 
vaso-motor  symptoms  are  more  pronounced  in  cold 
weather. 

Treatment. — The  general  princijDles  underlying  the 
treatment  of  peripheral  nerve  injuries  consist  chiefly  in 


122    DISEASES   OF  THE  NERVOUS   SYSTEM 

keeping  the  jjaralysed  muscles  in  a  position  of  relaxation, 
maintaining  their  nutrition  by  such  means  as  massage, 
electrical  treatment,  and  exercises,  and  giving  such 
drugs  as  may  be  necessary  for  the  relief  of  joain.  In 
cases  which  do  not  progress  satisfactorily  the  question  of 
operation  has  to  be  considered,  the  chief  indications  for 
which,  as  recently  laid  down  by  the  Medical  Research 
Council,*  are  (i.)  persistence  of  total  loss  of  conductivity 
(sensory  and  motor)  after  an  interval  of  two  months, 
although  proper  treatment  has  been  carried  out  ;  (ii.) 
palpable  neuromata  at  the  site  of  injury  of  a  nerve,  the 
function  of  which  is  seriously  disturbed  ;  (iii.)  when 
recovery  has  begun  but  has  not  progressed  at  the  usual 
rate  or  has  actually  ceased,  and  still  more  when  function 
has  relapsed;  (iv.)  persistent,  severe,  and  intractable 
pain.  On  the  other  hand,  operations  should  not  be 
undertaken  (a)  in  the  j^resence  of  an  unhealed  wound  or 
in  recently  healed  wounds  when  there  is  danger  of 
■  recrudescent  sepsis  ;  (6)  when  recovery  is  progressing 
(it  must  not  be  forgotten  that  early  signs  of  recovery 
may  be  associated  with  increased  discomfort) ;  (c)  when 
the  locality  of  the  injury  is  such  as  to  render  an 
operation  unusually  difficult  or  the  likelihood  of 
successful  suture  very  doubtful. 

The  circumflex  nerve  is  often  injured  from  lesions 
in  the  neighbourhood  of  the  shoulder- joint,  with  resulting 
paralysis  of  the  deltoid  and  teres  minor  together  with 
anaesthesia  of  an  area  of  skin  on  the  outer  aspect  of  the 
upper  arm  over  the  region  of  the  insertion  of  the  deltoid. 

In  dislocations  of  the  shoulder- joint  the  nerve  is 
often  stretched,  and  it  is  common,  even  after  the  dis- 
location has  been  reduced  quite  quickly,  to  find  some 
weakness  of  the  deltoid,  as  shown  by  inability  to  raise 
the  arm  to  the  hoiizontal  position  (Eig.  22).  In  other 
cases,  especially  those  of  subglenoid  dislocation,  the 
whole  plexus  may  be  severely  contused,  and  if  the  head 
of  the  bone  is  not  replaced  within  a  reasonable  time  the 

*  "  First  Report  of  the  Committee  upon  Injm-ies  of  the  Nervous 
System,"  1920.  To  this  the  reader  is  referred  for  further  details 
conceruiug  injuries  to  pcriiiheral  nerves. 


CIRCUMFLEX   NERVE   LESIONS  123 

results  may  be  disastrous.     In  such  cases  tlie  arm  is 
completely  paralysed,  the  muscles  waste  rapidly,  and 


Fig.  22.— Paralysis  of  the  deltoid.    Note  the  position  which  the 
patient  assumes  in  endeavouring  to  raise  his  arm. 

the  skin  assumes  the  characteristic  glossy  appearance 
There  is  usually  agonizing  pain,  with  varying  degrees  of 


124    DISEASES  OE  THE  NERVOUS  SYSTEM 

anaesthesia,  and  often  intense  liypersesthesia.  Massage 
and  electrical  treatment  must  be  given  regularly,  al- 
though at  first,  on  account  of  the  pain,  both  have 
frequently  to   be  administered  very  hghtly. 

For  the  pain,  local  sedatives  are  often  useful,  while, 
internally,  phenacetin  and  similar  drugs  are  beneficial. 
In  many  cases  morphia  is  required,  if  only  to  produce 
the  sleep  that  it  may  be  otherwise  impossible  to  obtain. 
In  one  case  in  which  the  pain  was  excruciating,  some 
relief  was  obtained  by  rapidly  alternating  applications 
of  heat  and  cold. 

Recovery  in  these  severe  cases  is  very  slow,  but 
may  in  the  end  be  complete,  provided  methods  of  treat- 
ment are  conscientiously  carried  out  by  the  patient  for 
a  long  time.  One  of  the  most  important  points  in  all 
cases  of  paralysis  of  the  deltoid  is  to  keep  the  injured 
muscle  in  a  state  of  relaxation,  which  can  be  done  by 
fixation  of  the  arm  in  a  position  of  abduction  by  means 
of  a  suitable  splint. 

The  pcctoralis  major,  which  obtains  its  nerve 
supply  from  the  external  and  internal  anterior  thoracic 
nerves,  is  an  important  muscle  to  examine.  It  consists 
of  an  upper  and  a  lower  division,  and  its  action,  which 
is  chiefly  to  adduct  the  arm,  can  be  shown  by  holding 
out  the  arms  horizontally  in  front  of  the  chest  and 
then  pressing  them  towards  the  middle  line  against 
resistance. 

The  lower  division  of  this  muscle  is  particularly 
liable  to  early  atrophy  in  pseudo-hypertrophic  paraly- 
sis ;   it  is  also  sometimes  absent  at  birth  (Fig.  23). 

The  long  or  posterior  thoracic  nerve  (nerve  of 
Bell). — Injury  or  disease  of  this  nerve  results  in  para- 
lysis of  the  serratus  magnus.  The  chief  functions  of 
this  important  muscle  are  (1)  to  fix  the  scapula,  so  that 
other  muscles  may  take  their  action  from  it,  and  (2)  to 
rotate  the  sca^iula  (in  conjunction  with  the  trapezius) 
when  the  arm  is  raised  above  the  shoulder. 

When  the  serratus  is  paralysed  the  scapula  can  no 
longer  be  fixed,  and  when  the  arm  is  raised  it  rotates 


PARALYSIS  OF  SEERATUS  MAGNUS   125 

so  that  its  vertebral  border  projects  and  presents  the 
wing-like  appearance  seen  in  the  illustration  (Fig.  24). 


Fig.  23.— Absence  (cougenital)  of  the  pectoralis  major  ou  the 
right  side.    {From  a  case  under  the  care  of  Mr.  T.  H.  Kellock.) 

Owing  to  this  want  of  fixation  the  power  of  pushing  is 
also  greatly  impaired.    The  diminished  power  of  rotating 


126    DISEASES   OF  THE   NERVOUS   SYSTEM 

the  scapula  is  seen  on  attempting  to  raise  the  arm 
above  the  level  of  the  shoulder,  which  movement,  in  the 
absence  of  the  serratus  magnus,  is  imperfectly  performed 
by  the  trapezius.     The  effects  of  paralysis  of  the  serratus 


Fig.  24.  Paralysis  of  the  serratus  magnus.  Note  the 
"  winging "  of  the  vertebral  border  of  the  scapula 
when  the  arm  is  raised. 


magnus  may  sometimes  be  confused  with  those  due  to 
paralysis  of  the  lower  part  of  the  trapezius.  The 
vertebral  border  of  the  scapula  stands  out  unduly  in 
both,  but,  in  the  absence  of  serratus  magnus  paralysis. 


SUBSCAPULAR    NERVE    LESIONS         127 

the  deformity  disappears  when  the  scapula  is  rotated 
by  raising  the  arm  above  the  head. 

There  may  also  be  some  difficulty  in  estimating  the 
power  of  the  serratus  magnus  when  the  deltoid  is  para- 
lysed, so  that  the  arm  cannot  be  raised  high  enough  to 
demonstrate  the  ability  or  inability  to  rotate  the  scapula. 
In  these  circumstances,  Duchenne  has  shown  that 
paralysis  of  the  serratus  magnus  can  be  demonstrated 
by  making  a  forward  movement  of  the  shoulder  :  if 
the  muscle  is  acting,  the  lower  angle  of  the  scapula  is 
moved  forwards  and  outwards ;  while  if  it  is  paralysed, 
the  spinal  border  does  not  move  except  to  become 
rather  more  prominent  through  some  rotation  on  its 
vertical  axis. 

Affections  of  the  long  subscapular  nerve  result 
in  paralysis  of  the  latissimus  dorsi.  Contraction  of 
this  muscle  can  be  shown  by  adducting  and  depressing 
the  arm  against  resistance. 

C.  E.  Beevor*  showed  that  while  only  one  latissimus 
dorsi  acts  in  a  voluntary  adductor  movement  of  the 
arm,  the  two  muscles  act  together  in  such  involuntary 
movements  as  coughing  and  sneezing.  In  cases  of 
hemiplegia  it  may  be  possible  to  show  loss  of  power 
of  the  muscle  to  perform  voluntary  movements  while 
those  for  involuntary  ones  are  preserved,  just  as,  in 
the  face  of  a  hemiplegic,  the  corner  of  the  mouth  mil 
not  move  voluntarily,  but  will  do  so  in  the  expression 
of  emotions  such  as  laughing  and  crying. 

Beevor  suggested  that  a  knowledge  of  this  double 
action  might  at  times  be  useful  in  differentiating  diseases 
of  the  brain  from  those  of  the  cord  and  peripheral  nerves, 
for  whereas  in  cerebral  lesions  the  involuntary  bilateral 
movements  might  be  preserved,  in  trouble  at  the  peri- 
phery all  movements  would  be  lost.  He  further  showed 
that,   though  the  latissimus  has  generally  been   con- 

*  Brit,  Mod.  Journ.,  1898,  ii.  976.     As  pointed  out  by  Hughlings 
Jackson,  the  upper  part  of  the  trapezius  acts  in  a  similar  manner ;    ' 
the  highest  fibres  act  unilaterally  to  elevate  the  shoulder  and  bilater* 
ally  wheii  called  into  play  to  assist  in  respiratioi).. 


128   DISEASES   OF   THE   NERVOUS   SYSTEM 

sidered  to  be  a  muscle  of  inspiration,  its  chief  action  is  in 
reality  expiratory  in  character. 

The  musculo-cutaneous  nerve  arises  from  the 
outer  cord  of  the  brachial  plexus  and  supplies  the  coraco- 
brachialis,  the  biceps  and  brachialis  anticus.  It  supjilies 
sensation  to  the  outer  side  of  the  forearm,  but  its  dis- 
tribution is  variable,  and  the  resulting  changes  after 
injury  are  usually  indefinite  in  character. 

The  museulo-spiral  nerve,  the  longest  offshoot 
from  the  brachial  plexus,  is  derived  from  the  posterior 
cord.  At  first  situated  behind  the  third  part  of  the 
axillary  artery  and  the  commencement  of  the  brachial, 
it  then  winds  round  the  back  of  the  humerus  in  the 
museulo-spiral  groove  between  the  external  and  in- 
ternal heads  of  the  triceps,  and  appears  upon  the  outer 
aspect  of  the  arm,  between  the  brachialis  anticus  on 
the  inside  and  the  supinator  longus  and  the  extensor 
carpi  radialis  longior  on  the  outside  ;.  opposite  the  ex- 
ternal condyle  of  the  humerus  it  divides  into  the  pos- 
terior interosseous  and  the  radial  nerve.  The  museulo- 
spiral  nerve  supplies  the  three  heads  of  the  triceps,  the 
supinator  longus,  the  extensor  carpi  radialis  longior, 
the  brachialis  anticus  (in  part),  the  anconeus,  the  sub- 
anconeus,  and,  through  its  posterior  interosseous  branch, 
to  the  extensor  carpi  radialis  brevior,  supinator  brevis, 
extensor  communis  digitorum,  extensor  minimi  digiti, 
extensor  carpi  ulnaris,  extensor  indicis,  and  the  extensors 
of  the  thumb. 

The  museulo-spiral  nerve  may  be  injured  by  pene- 
trating wounds  from  bullets  or  other  weapons.  It  is 
also  apt  to  be  compressed  by  callus  resulting  from 
a  fracture  of  the  shaft  of  the  humerus,  especially  in  the 
neighbourhood  of  the  museulo-spiral  groove.  On  the 
inner  side  of  the  arm  the  nerve  may  be  injured  by 
the  pressure  of  crutches  or  by  the  pressure  induced  by 
the  arm  hanging  over  a  chair  or  other  hard,  resisting 
object,  especially  if  the  patient  is  under  the  influence  of 
alcohol  or  other  narcotic.  The  wrist-drop  which  some- 
times  occurs   after  the   arm  has   been  lain   on  during 


EADIAL   NERVE  129 

heavy  sleep  is  generally  attributed  to  injury  of  the 
nerve,  but  it  seems  probable,  as  suggested  by  Sir  Robert 
Jones,  that  in  many  such  cases  the  paralysis  may  be 
due  to  overstretching  of  muscles  rather  than  to  injury 
of  the  nerve. 

The  fibres  of  the  nerve,  with  the  exception  of  those 
going  to  the  supinator  longus,  are  especially  liable  to  be 
affected  in  chronic  lead-poisoning.     {See  p.  156.) 

The  chief  results  of  injury  to  the  musculo-spiral 
nerve  are  loss  of  power  in  the  triceps  and  supinator 
longus,  together  with  weakness  of  the  extensor  muscles 
of  the  forearm,  giving  rise  to  wrist-drop  and  weakness  of 
extension  of  the  fingers  at  the  metacarpo-phalangeal 
joints,  the  signs  difiering  according  to  the  level  of  the 
lesion. 

The  posterior  interosseous  nerve  winds  round 
the  neck  of  the  radius  in  the  substance  of  the  supinator 
brevis  (which  it  supplies)  to  reach  the  back  of  the  fore- 
arm, and  then  runs  down  between  the  superficial  and 
deep  extensors  on  the  back  of  the  forearm ;  these  it 
innervates,  as  described  on  p.  128. 

The  radial  nerve  is  purely  sensory,  and  is  dis- 
tributed to  the  dorsal  aspect  of  the  thumb  as  far  as  the 
bed  of  the  nail,  and  to  a  varying  extent  of  surface  on  the 
back  of  the  index,  middle,  and  ring  fingers.  The  over- 
,  lapping  of  this  nerve  with  the  external  cutaneous  branch 
of  the  musculo-spiral  is,  however,  such  that  no  definite 
anaesthesia  usually  follows  a  lesion  of  the  radial  unless 
the  external  cutaneous  branch  is  at  the  same  time 
affected. 

The  prognosis  depends  on  the  nature  and  degree 
of  injury.  Paralysis  from  temporary  pressure  on  the 
musculo-spiral  trunk  is  usually  favourable.  A  few 
days  or  a  week  generally  suffices  to  restore  power, 
but  sometimes  a  much  longer  period  is  required,  and 
recovery  appears  to  be  a  distinctly  slower  process  in 
elderly  people.  During  treatment  care  must  be  taken 
to  keep  the  paralysed  muscles  of  the  forearm  relaxed  by 
extension  of  the  wrist  and  fingers  at  the  metacarpo- 
J 


130    DISEASES   OF   THE   NERVOUS   SYSTEM 

phalangeal  joints  by  means  of  a  sj^lint  for  the  wrist  and 
elastic  extension  for  the  fingers  and  thumb.  The  outlook 
after  suture  of  the  musculo-spiral  nerve  is  good.  '*** 

The  ulnar  nerve,  arising  from  the  inner  cord  of 
the  brachial  plexus,  runs  along  the  inner  side  of  the 
arm,  and  enters  the  forearm  by  passing  between  the 
internal  condyle  of  the  humerus  and  the  olecranon. 
In  the  forearm  it  runs  down  upon  the  surface  of  the 
flexor  profundus  digitorum  between  the  flexor  carpi 
ulnaris  on  the  inside  and  the  flexor  sublimis  digitorum 


Median. 

Fig.  25.— Distribution  of  the  sensory  nerves  in  the  hand. 
{^AUev  Bernhardt.) 


on  the  radial  side  ;  in  the  lower  two-thirds  of  the  fore- 
arm it  is  closely  associated  with  the  ulnar  artery,  lying 
upon  the  ulnar  side  of  the  vessel.  It  passes  in  front  of 
the  anterior  annular  ligament  and  divides  into  a  super- 
ficial and  a  deep  portion.  In  the  forearm  it  innervates 
the  flexor  carpi  ulnaris  and  the  ulnar  half  of  the  flexor 
profundus  digitorum,  and  it  often  gives  of!  its  dorsal 
cutaneous  branch  as  high  as  the  middle  third  of  the 
forearm,  which  supplies  the  ulnar  portion  of  the  dorsum 
of  the  hand,  and  the  dorsal  aspect  of  the  inner  one  and 
a  half  digits  as  far  as  the  middle  of  the  phalanges. 


ULNAR   PARALYSIS  131 

The  nerve  also  supplies  most  of  the  intrinsic  muscles 
of  the  palm,  namely,  the  seven  interossei,  the  two  inner 
lumbricals,  the  three  muscles  of  the  hypothenar  emi- 
nence, the  two  adductors  of  the  thumb,  and  the  deep 
head  of  the  flexor  brevis  pollicis  ;  it  supplies,  too,  the 
skin  over  the  palmar  aspect  of  the  inner  one  and  a  half 
digits,  sending  branches  on  to  the  dorsum  as  far  up  as 
the  middle  and  second  phalanges.     (Fig.  25.) 

The  results  of  ulnar  paralysis  (Fig.  26)  are  loss  of 
power,  with  wasting  of  the  muscles  mentioned  above. 
Weakness  of  the  interossei  shows  itself  by  inability  to 


Fig.  28.— Ulnai'  paralysis.  Note  the  "  claw-hand "  shape,  and 
the  hollow  caused  by  the  wasting  of  the  first  interosseous 
(abductor  indicis). 

separate  the  fingers  in  the  horizontal  plane,  and  th® 
wasting  becomes  obvious  from  the  hollows  between  th^ 
metacarpal  bones,  especially  the  concavity  of  the  radial 
side  of  the  first  metacarpal,  due  to  loss  of  substance  of 
the  first  dorsal  interosseous  (the  abductor  indicis).  The 
slight  degree  of  abduction  of  the  index  and  little  fingers 
which  can  still  be  performed  by  the  extensors  and  lum- 
bricals must  not  be  mistaken  for  the  action  of  the 
interossei. 

Loss  of  adduction  of  the  thumb,  i.e.  the  movement 
of  the  thumb  which  brings  its  ulnar  border  alongside 
the  metacarpal  bone  of  the  first  finger,  can  be  shown 
by  asking  the  patient  to  press  something  between  the 


132   DISEASES   OF  THE  NERVOUS  SYSTEM 

ulnar  border  of  the  thumb  and  the  first  finger.  Weak- 
ness of  this  movement  indicates  loss  of  power  of  the 
adductor  and  inner  head  of  the  flexor  brevis  pollicis, 
and  a  hollow  over  the  site  of  these  muscles  is  often 
visible.  Abduction  of  the  index  and  little  fingers  can 
be  performed  to  a  limited  extent  by  their  extensor 
tendons,  and  the  contraction  of  the  first  lumbrical  in 
the  first  space  may  be  mistaken  for  that  of   the    ab- 


Fig.  27.— Loss  of  sensibility  after  complete  division  of  the 
ulnar  nerve.    {Sherren,  in  Choyce's  System  of  S^irciery.)* 

ductor  indicis.  After  paralysis  has  lasted  for  some  time 
the  hand  tends  to  assume  the  claw  position  {main- 
en-grijje),  which  must  be  distinguished  from  Volkmann's 
contracture  {see  p.  136). 

The  extent  to  which  anaesthesia  is  produced  by 
injuries  of  the  ulnar  nerve  differs  according  as  the 
lesion  is  situated  above  or  below  the  origin  of  the  dorsal 
cutaneous    branch.     When    the    injury    is    above    the 

*  In  this  diagram,  and  in  Figs.  28  and  29,  the  area  included  within 
the  firm  line  is  that  of  insensibility  to  light  touch ;  the  shaded  area, 
that  of  insensibility  to  prick  as  vrell  as  to  light  touch. 


ULNAH   PAEALYSIS 


133 


origin  of  the  dorsal  brancli  the  loss  of  sensation  is  dis- 
tributed over  the  ulnar  side  of  the  hand,  including  the 
whole  of  the  little  and  half  the  ring  fingers  (Fig.  27). 
When,  however,  the  nerve  is  injured  just  above  the 
wrist — ^the  most  frequent  site  of  injury — ^the  dorsal 
branch  escapes,  and  the  extent  of  the  anaesthesia  will 
consequently  be  much  less,  the  patient  retaining  sensa- 
tion over  the  proximal  parts  of  the  dorsal  aspects  of 


Fig.  28.— Loss  of  sensibility  following  division  of  the  ulnar 
nerve  below  its  dorsal  branch.  {Sherren,  in  Choyce's 
Si/stem  of  Surgery.) 

Sec  footnote  on  p.  132. 


the  ring  and  little  fingers  while  the  peripheral  por- 
tions of  these  digits  are  anaesthetic  (Fig.  28).  In  both 
cases  the  area  of  anaesthesia,  as  seen  in  the  figures,  is 
more  extensive  to  light  touch  than  to  painful  stimuH, 
owing  to  the  overlapping  which  takes  place  in  the 
fibres  for  the  latter. 

The  impulses  for  deep  sensibility  are  conveyed  along 
the  nerves  of  muscles  and  tendons,  and  unless  these  latter 
are  injured  the  patient  will  be  able  to  recognize  the 


134     DISEASES   OF  THE  NERVOUS   SYSTEM 

sensations  of  passive  positions,  deep  pressure,  and  the 
vibrations  of  a  tuning-fork. 

The  median  nerve,  being  less  exposed,  is  less  liable 
to  injury  than  the  ulnar,  but  may  be  damaged  in  fractures 
and  dislocations  of  the  humerus.  It  is  most  frequently 
injured  above  the  wrist  in  such  accidents  as  the  bursting 
of  bottles  or  thrusting  the  arm  through  a  window. 

The  median  nerve  arises  by  two  heads,  one  from  the 
outer  and  the  other  from  the  inner  cord  of  the  brachial 
plexus.  The  nerve  is  first  situated  on  the  outer  side  of 
the  axillary  and  the  brachial  arteries,  but  in  the  middle 
oE  the  arm  it  crosses  the  latter  vessel  from  without 
inwards,  and  runs  down  on  the  inner  side  as  far  as  the 
elbow.  It  passes  between  the  two  heads  of  the  pro- 
nator radii  teres,  being  separated  from  the  ulnar  artery 
by  the  deep  head  of  the  latter  muscle,  and  then  con* 
tinues  straight  down  the  forearm  between  the  flexor 
sublimis  and  flexor  profundus  digitorum  muscles  ;  just 
above  the  anterior  annular  ligament  it  lies  exactly 
posterior  to  the  palmaris  longus  tendon,  if  this  is  present. 
It  passes  behind  the  anterior  annular  ligament  enveloped 
by  the  synovial  sheath  which  is  wrapped  round  the  ten- 
dons of  the  flexors  of  the  fingers,  and,  reaching  the  palm, 
divides  to  supply  the  muscles  of  the  thenar  eminence, 
viz.  the  abductor  polUcis,  the  opponens  pollicis,  and  the 
superficial  head  of  the  flexor  brevis  pollicis.  It  inner- 
vates the  two  outer  lumbricals,  and  supplies  the  skin 
over  the  palmar  aspect  of  the  radial  side  of  the  hand, 
over  the  front  of  the  thumb,  the  index,  middle,  and 
half  the  ring  fingers,  and  over  varying  portions  of  the 
dorsum  of  the  same. 

In  the  forearm  the  median  supplies  all  the  muscles 
arising  from  the  internal  condyle,  except  the  flexor 
carpi  ulnaris  ;  through  its  anterior  interosseous  branch 
it  innervates  the  deep  muscles  of  the  forearm,  viz.  the 
flexor  longus  pollicis,  the  pronator  quadratus,  and  the 
outer  half  of  the  flexor  profundus  digitorum  (the  inner 
half  of  the  latter  muscle  deriving  its  nerve  supply  from 
the  ulnar  nerve). 


MEDIAN   NERVE  LESIONS 


135 


In  cases  of  lesion  of  the  median  nerve  there  will  be 
loss  of  power  in  the  muscles  supplied  by  the  nerve, 
and  anaesthesia  over  the  area  of  skin  which  it  innervates  ; 
but  the  action  of  some  of  the  muscles  to  which  the 
median  is  distributed  may  be  closely  simulated  by  others 
drawing  their  nerve  supply  from  a  different  source. 
Flexion  of  the  wrist,  for  instance,  can  be  j)erformed  by 


Fig.  29.— Loss  of  sensibility  following  division  of  the  median 
nerve.    (Sherren,  in  Choijce's  System  of  Svrqerij.) 

See  footnote  on  p.  132. 


ulnar  muscles,  and  also  to  some  extent  by  the  extensor 
ossis  metacarpi  pollicis. 

Action  of  the  abductor  breads  pollicis  can  be  closely 
simulated  by  that  of  the  extensor  ossis  metacarpi  pollicis, 
but,  as  Beevdr  pointed  out,  true  abduction  of  the  thumb 
takes  place  in  a  plane  at  right  angles  to  that  of  the  palm, 
and  abduction  by  means  of  the  extensor  ossis  metacarpi 
pollicis  is  always  comjilicated  by  extension  of  the  thumb 
at  the  same  time. 

Sensation  to  light  touch  is  abolished  over  the  area 
of  skin  supplied  by  the  nerve,  but  the  extent  to  which 


136    DISEASES  OP  THE  NERVOUS  SYSTEM 

sensation  to  pin-prick  is  lost  varies,  and  is  usually  much 
less  than  that  of  touch.  (Fig.  29.)  Presence  or  loss  of 
deep  sensibility  will  depend  upon  the  integrity  or  other- 
wise of  the  tendons  along  which  the  nerves  for  this  form 
of  sensation  run.- 

Volkmann's  Conteacture,  or  Ischemic  Paralysis 

This  form  of  paralysis  was  first  described  by  Volk- 
mann  in  1875,  and  is  characterized  by  a  contraction 
of  the  forearm  muscles,  which  causes  contraction  of 
the  fingers  and  sometimes  also  of  the  wrist. 

Etiology. — The  affection  usually  arises  during 
treatment  for  fracture  in  the  region  of  the  elbow-joint, 
especially  in  connection  with  fractures  of  the  lower  end 
of  the  humerus,  or  a  separation  of  the  lower  epiphysis 
of  that  bone.  Undue  pressure  hy  splints  and  tight  band- 
aging appears  to  play  the  most  important  part  in  the 
production  of  the  contracture,  as  is  evidenced  by  the 
fact  that  in  a  large  number  of  the  cases  the  splints 
exercised  sufficient  pressure  to  cause  sloughing.  It  is 
not  so  much  that  the  splint  is  applied  too  tightly  at 
first,  as  that  no  allowance  is  made  for  the  swelling  that 
is  likely  to  ensue  in  the  next  few  days. 

Prolonged  obstruction  to  the  circulation  by  an 
Esmarch's  bandage,  injury  to  the  brachial  artery,  rup- 
ture or  severe  contusion  of  the  flexors,  and  prolonged 
suppuration  in  the  muscles  have  also  been  assigned 
as  causes  of  the  contracture. 

The  above  condition  is  most  frequently  met  with  in 
children. 

Symptoins. — The  chief  characteristics  are  hard- 
ness of  the  muscles,  followed  by  contraction  of  the  flexors 
of  the  forearm,  causing  flexion  of  the  fingers,  and  some- 
times of  the  wrist ;  and  in  severe  cases  the  forearm  is 
fully  pronated  and  fixed  in  this  position.  When  the 
wrist  is  flexed  it  may  be  possible  to  uncoil  the  fingers  to 
a  varying  degree,  but  as  soon  as  the  wrist  is  extended 
the  pull  of  the  tightened  flexors  is  felt,  and  the  hand 
again  assumes  a  claw-like  aspect,  the  metacarpo-phalan- 


ISCHEMIC   PARALYSIS  137 

geal  joints  being  in  a  condition  of  hyperextensicn  and 
the  middle  and  terminal  phalanges  clenched  in  extreme 
flexion.     (Figs.  30,  31.) 

Pathology. — The  exact  nature  of  the  condition  is  not 
quite  clear,  and  the  term  ''  ischsemic  "  has  been  apphed 
on  the  supposition  that  the  pressure  of  the  splints 
induces  the  changes  by  arresting  the  circulation  in  the 
muscles.  The  rapidity  with  which  the  symptoms  show 
themselves,  together  with  the  fact  that  in  some  cases 
there  are  no  alterations  in  the  electrical  reactions  and 
no  anaesthesia,  supports  the  hypothesis  that  the  lesion 
is  primarily  one  of  muscular  rather  than  of  nervous 
origin.     The  pale,  firm,  fibroid  condition  of  the  muscles 


Fig.  30.— Position  of  baud  in  an  early  stage  of  ischsemic 
paralysis. 

is  also  in  favour  of  this  view,  and  the  very  early  onset 
of  the  contracture  in  some  cases,  supervening  even  in 
a  few  hours,  suggests  the  presence  of  some  coagulation 
process  or  local  "  rigor  mortis  "  of  the  compressed  fibres 
as  the  pathological  condition  rather  than  a  myositis, 
though  this  very  early  onset  may  possibly  be  due  to 
nerve  spasm.  On  the  whole,  an  inflammatory  con- 
dition of  the  muscular  fibres  seems  the  most  likely 
cause  of  the  contracture. 

In  some  cases  electrical  changes  and  anaesthesia 
have  been  observed,  but  this  fact  does  not  necessarily 
invalidate  the  hypothesis  that  the  disease  is  of  muscular 
origin,  for  it  is  quite  possible  for  injuries  to  the  nerves 
to   coexist   with   Volkmann's    contracture,   and   some- 


138    DISEASES   OF   THE   NERVOUS   SYSTEM 

times  the  median  and  ulnar  nerves  have  been  found 
embedded  in  scar  tissue,  but  otherwise  healthy  in 
appearance. 

Diagnosis. — The  diagnosis  is  based  upon  the 
history,  the  character  of  the  deformity,  the  absence 
of   electrical    changes,   the   frequent    absence   of   anses- 


Fig.  31.— Position  of  fingers  caused  by  contraction 
of  the  flexor  tendons  in  ischemic  paralysis. 

thesia,  and  the  simultaneous  onset  of  paralysis  and 
contracture. 

Ulnar  paralysis,  in  its  later  stages,  to  a  certain 
extent  resembles  Volkmann's  contracture,  but  in  the 
former  there  is  no  flexion  of  the  wrist,  while  the  small 
muscles  of  the  hand  (except  those  supplied  by  the 
median)  are  wasted  and  show  the  reaction  of  degenera- 
tion. Further,  there  may  be  anaesthesia  or  paraesthesia 
over  the  area  of  skin  supplied  by  the  ulnar  nerve. 

Late  musculo- spiral  paralysis. — In  this  condition  the 
extensors  of  the  wrist  and  fingers  are  paralysed  and 


ISCHEMIC   PARALYSIS  139 

give  the  reaction  of  degeneration.  The  supinator  longus 
and  triceps  may  share  in  the  paralysis,  and  the  ter- 
minal phalanges  can  still  be  extended  by  the  interossei 
and  lumbricales. 

Infantile  paralysis  (acute  poliomyelitis)  is  distin- 
guished from  Volkmann's  contracture  by  the  history, 
the  presence  of  the  reaction  of  degeneration,  and  the  fact 
that  contracture  follows  the  paralysis  at  a  later  date. 

Treatment. — Massage,  passive  movements,  and 
electricity  may  lead  to  much  improvement  in  mild 
cases,  but  they  must  be  commenced  early  and  practised 
regularly.  Even  in  the  severe  cases,  although  these 
measures  fail  to  cure  the  deformity,  they  are  neverthe- 
less of  value  in  maintaining  the  nutrition  of  the  muscles, 
and  should  be  given  a  fair  trial  for  three  or  four  months 
before  any  treatment  by  operation  is  considered. 

Prognosis. — Some  mild  cases  may  recover  by  the 
systematic  employment  of  active  and  passive  move- 
ments, massage,  and  electricity,  but  in  many  cases 
operation  is  necessary  to  permit  of  a  more  extensive 
range  of  movement  for  the  diseased  muscles,  and  to 
correct  the  deformity,  before  the  former  method  of 
treatment  can  be  of  any  permanent  value. 


CHAPTER   XIII 

PARALYSES    OF   THE   LOWER    LIMB 

The  lumbar  and  sacral  plexuses,  like  the  brachial,  may 
suffer  from  pressure,  and  the  effects  of  paralyses  of  the 
lower  limb  are  likewise  differently  distributed  according 
as  the  lesions  affect  the  roots  or  the  peripheral  nerves. 
The  following  table,  taken  from  Morris's  "Anatomy," 
shows  the  relations  of  the  muscles  to  the  roots  of  the 
lumbar  and  sacral  plexuses  : — 

NERVE    ROOTS  MUSCLES 

2nd  and  3rd  L Ilio-psoas. 

Sartorius. 
Peclineus. 
Adductor  longus. 

2nd,  3rd,  and  4th  L, .     .     .     Gracilis. 

Adductor  brevis. 

ord  and  4tli  L Quadriceps  extensor. 

Obturator  extern  us. 

3rd,  4th,  and  5th  L.  .     .     .     Adductor  mag'nus. 

4th,  5th  L.,  and  1st  S.    .     .     Gluteus  medius. 

Gluteus  minimus. 
Tensor  fasc.  femoris. 
Femimembranosus. 
Plantaris. 
Popliteus. 
(^uadratus  femoris. 
Inferior  g-omellus. 

5th  L.  and  1st  S Flexor  long,  digit. 

Tibialis  posticus. 
Flexor  brcv.  digit. 
Flexor  brcv.  haliucis. 
Abductor  haliucis. 
First  lumbrical. 

]40 


PARALYSES   OF  LOWER  LIMB  141 

NERVE    ROOTS  MUSCLES 

oth  L.,  1st  and  '2nd  S.       .     .     Superior  gemellus. 

Obturator  internus. 
Gluteus  maximus. 
Kemitendinosus. 
Soleus. 
Flexor  long,  hallucis. 

1st  and  2nd  S Pyriformis. 

Gastrocnemius. 
Flexor  accessorius. 
Abd.  min.  digiti. 
Plantar  interossei. 
Dorsal  interossei. 
Adductor  hallucis  trans. 
Adductor  hallucis  obliq. 

1st,  2nd,  and  3rd  S.     .     .     .     Long  head  of  biceps. 

Ext.  long,  hallucis. 
Ext.  long,  digit. 
Ext.  brev.  digit. 

4th,  5th  L.,  and  1st  S.      .     .     Tibialis  anticus. 

Peroneus  tertius,  longus, 
bra  vis. 

The  ilio-inguinal  and  ilio-hypogastrie  nerves 

arise  from  the  1st  lumbar  root,  and,  together  with  the 
genito-crural  nerve,  which  arises  from  the  1st  and 
2nd  roots,  terminate  by  supplying  the  skin  over  the 
upper  part  of  the  thigh. 

The  obturator  nerve  arises  from  the  2nd,  3rd,  and 
4:th  lumbar  nerves.  From  its  branches  the  adductor 
longus,  adductor  brevis,  adductor  magnus,  gracilis,  obtu- 
rator externus,  and  (rarely)  the  pectineus  derive  their 
nerve  supply. 

When  this  nerve  is  paralysed  there  is  partial  loss  of 
power  to  adduct  the  thigh.  The  adductor  magnus 
still  retains  some  power,  since  it  is  partially  supplied 
from  the  great  sciatic  nerve,  and  adduction  may  also 
be  assisted  by  the  pectineus,  which  often  derives  some 
of  its  supply  from  an  accessory  obturator  nerve. 

The  external  cutaneous  nerve  arises  from  the 
2nd  and  3rd  lumbar  roots.  It  supplies  the  skin  over 
the  outer  part  of  the  thigh  with  sensation.     Tingling 


Hii    DISEASES   OF   THE   NERVOUS   SYSTEM 

and  parsesthesia  of  various  kinds  are  apt  to  occur  in  the 
distribution  of  this  nerve. 

The  anterior  crural  nerve  springs  from  the  2nd, 
3rd,  and  4th  lumbar  roots,  which  unite  to  form  one 
trunk  between  the  psoas  and  ihacus  muscles.  Besides 
sending  branches  to  the  iliacus,  pectineus,  and  sartorius, 
it  also  supplies  the  quadriceps  extensor,  which  is  com- 
posed of  the  rectus,  vasti,  and  crureus.  The  middle 
and  internal  cutaneous  nerves,  which  supply  the  skin 
over  the  lower  two-thirds  of  the  anterior  and  inner 
surfaces  of  the  thigh,  and  the  long  saphenous  nerve, 
which  supplies  sensation  over  the  anterior  and  inner 
part  of  the  leg,  all  arise  from  the  anterior  crural. 

The  chief  motor  result  of  disease  of  the  anterior 
crural  nerve  is  loss  of  power  to  extend  the  knee,  from 
paralysis  of  the  quadriceps  extensor.  The  knee-jerk 
is  diminished  or  lost.  Flexion  of  the  hip  is  weakened 
through  paralysis  of  the  iliacus,  but  it  can  still  be  carried 
out  by  the  psoas,  which  is  supplied  by  special  branches 
from  the  2nd  and  3rd  lumbar  nerves.  Sensation  may 
be  impaired  over  those  parts  of  the  leg  and  thigh 
which  the  nerve  suppUes. 

The  superior  gluteal  nerve  arises  from  the  lumbo- 
sacral cord  and  the  1st  sacral  nerve.  It  is  responsible 
for  the  nerve  supply  of  the  gluteus  medius  and  minimus, 
and  of  the  tensor  vaginae  femoris. 

The  inferior  gluteal  nerve  supplies  the  gluteus 
maximus.  The  gluteus  maximus  is  one  of  the  principal 
extensors  of  the  thigh,  but  is  also  an  abductor  and  out- 
ward rotator.  Its  action  can  be  tested  by  asking  the 
patient  to  lie  flat  on  his  face  and  then  to  raise  his  thigh. 
The  gluteus  maximus  is  also  of  importance  from  the 
frequency  with  which  it  is  enlarged  in  pseudo-hyper- 
trophic  paralysis.  The  gluteus  medius  and  minimus 
are  both  abductors,  but  they  also  to  some  extent  flex 
and  rotate  the  thigh  inwards. 

The  small  sciatic  nerve  most  usually  arises  from 
the  1st,  2nd,  and  3rd  sacral  roots.  After  running  down 
the  back  of  the  thigh  and  giving  off  branches  which 


SCIATIC  NERVE   PAEALYSIS  143 

supply  the  skin  over  the  back  of  the  thigh  and  popliteal 
space,  it  pierces  the  deep  fascia  of  the  leg  below  the  knee 
and  supplies  the  skin  over  the  upper  part  of  the  calf. 

Great  Sciatic  Nerve  and  its  Branches 
The  great  sciatic  nerve  arises  from  the  sacral  plexus 
at  the  lower  border  of  the  great  sacro-sciatic  foramen. 
It  runs  down  the  back  of  the  thigh  and  terminates  by 
dividing  into  the  external  and  internal  popliteal  nerves. 
As  the  great  sciatic  nerve  passes  down  the  thigh, 
branches  leave  it  to  supply  the  biceps  femoris,  semimem- 
branosus, semitendinosus,  and  a  portion  of  the  adductor 
magnus,  which  may  all  suffer  in  severe  cases  of  sciatica. 
Paralysis  of  the   sciatic  nerve  may  be  caused 
by  injuries  of  different  kinds,  and  the  nerve  trunk  may 
be  divided  by  stabs  or  bullet  wounds.     In  this  connec- 
tion it  is  important  to  remember  that  the  two  main 
divisions  of  the  nerve,  i.e.  the   internal    and    external 
popliteal    branches,    although    contained    in    the    one 
sheath,  remain  separate  from  one  another  up  to  their 
origin  from  the  sacral  plexus.     It  is  therefore  possible 
in  injuries   of  the  main  trunk  for   one  di^dsion  to   be 
damaged   while  the   other   escapes.     Complete   division 
of  the  sciatic  nerve  is  followed  by  loss  of  power  in  all 
the  muscles  which  it  supplies,  and  loss  of  sensation  over 
a  considerable  portion  of  the  foot  and  leg,  as  shown  in 
Fig.  32. 

At  the  top  of  the  popliteal  space  the  main  trunk 
divides  into  external  and  internal  pophteal  nerves. 

The  external  popliteal  nerv^e  (peroneal  nerve) 
descends  obliquely  along  the  outer  side  of  the  popliteal 
space,  running  close  to  the  biceps  tendon.  It  then  winds 
round  the  neck  of  the  fibula  (in  which  position  it  is  very 
liable  to  injury),  and,  passing  between  the  latter  and  the 
peroneus  longus,  divides  into  the  anterior  tibial  and 
the  musculo-cutaneous  nerves. 

The  external  popliteal  nerve  may  be  injured  in 
punctured  wounds  of  the  upper  part  of  the  popliteal 
space  or  at  the  neck  of  the  fibula,  and  also  when  the 


144    DISEASES   OF   THE   NERVOUS   SYSTEM 

biceps  tendon  is  divided  subcutaneously.  The  peronei, 
tibialis  anticus,  and  extensors  of  the  toes  will  be  para- 
lysed, and  there  will  be  loss  of  sensation  over  the  area 
of  skin  supplied  by  its  two  divisions,  the  anterior  tibial 
and  musculo-cutaneous  nerves. 

The  anterior  tibial  nerve,  commencing  between 
the  fibula  and  the  peroneus  longus,  inclines  obliquely 
beneath  the  extensor  longus  digitorum  to  reach  the 
anterior   surface    of   the    interosseous    membrane.      It 


'^  Fig.  32.— Effects  of  complete  division  of  the  great  sciatic 
nerve.  The  area  of  loss  of  sensibility  to  light  touch  is 
bounded  by  a  thin  line  ;  that  to  pi'ick,  by  crosses ;  that 
to  all  forms  of  sensibility,  including  deep  touch,  by  a 
thick  line.    {Sherren,  in  Choijce's  System  of  Surgenj.) 

runs  down  the  front  of  the  limb  till  it  reaches  the  level 
of  the  ankle-joint,  where  it  divides  into  internal  and 
external  branches,  the  former  supplying  the  integument 
on  the  neighbouring  sides  of  the  great  toe  and  the  second 
toe  on  their  dorsal  aspect,  the  latter  being  distributed 
partly  as  motor  branch  to  the  extensor  brevis  digi- 
torum, and  partly  as  sensory  twigs  to  the  dorsal  inter- 
ossei.  In  the  leg  the  anterior  tibial  supplies  the  tibialis 
anticus,  extensor  proprius  hallucis,  extonsor  longus  digi- 
torum, and  peroneus  tertius. 


MUSCULO-CUTANEOUS  NERVE  145 

Paralysis  of  the  anterior  tibial  nerve  leads  to  joot- 
drop.  The  foot  cannot  be  flexed  on  the  leg,  and  the 
difficulty  in  walking,  consequent  on  the  toes  catching 
in  the  ground,  gives  rise  to  a  "  high-stepping  "  gait. 

The  musculo-cutaneous  nerve,  lying  in  a  sheath 
in  the  intermuscular  septum  between  the  peronei  ex- 
ternally and  the  extensor  longus  digitcrum  internally, 
runs  down  in  front  of  the  fibula  to  the  lower  third  of  the 
leg,  where  it  pierces  the  deep  fascia  in  two  branches, 
internal  and  external.  The  main  trunk  innervates  the 
peroneus  longus  and  brevis,  and  of  the  two  terminals 
the  internal  branch  comes  down  over  the  anterior  an- 
nular ligament  and  supplies  the  lower  third  of  the  leg, 
the  skin  over  the  dorsum  of  the  foot,  the  inner  side  of  the 
big  toe,  and  the  adjacent  side  of  the  second  and  third 
toes.  The  external  branch  supplies  the  adjacent  sides 
of  the  third  and  fourth,  and  fourth  and  fifth  toes,  com- 
municating with  the  external  saphenous  nerve.  The 
action  of  the  peroneus  longus  and  brevis  is  to  evert 
the  foot  and  also  to  aid  in  extending  the  ankle-joint. 

When  these  muscles  are  paralysed  the  power  of 
e version  is  lost,  and  the  foot  is  turned  inwards  by  the 
unantagonized  action  of  the  tibialis  anticus  and  posticus. 

The  internal  popliteal  passes  onwards  through 
the  popliteal  space,  and  at  the  lower  border  of  the 
popliteus  becomes  continuous  with  the  posterior  tibial 
nerve.  The  latter  runs  down  the  back  of  the  leg  and 
terminates  in  the  internal  and  external  plantar 
nerves*  The  internal  popliteal  and  posterior  tibial 
nerves  supply  the  gastrocnemius,  soleus,  plantaris, 
popliteus,  tibialis  posticus,  flexor  longus  digitorum,  and 
flexor  longus  hallucis. 

In  paralysis  of  these  muscles  there  is  loss  of  power 
to  extend  the  foot  on  the  ankle-joint  and  to  flex  the 
toes.  In  long-standing  cases  the  unantagonized  action 
of  the  flexors  produces  a  condition  of  talipes  calcaneus, 
in  which  only  the  heel  can  be  placed  on  the  ground. 

The  internal  plantar  nerve  is  mainly  sensory,  sup- 
plying the  palmar  surface  of  the  inner  three  and  a  half 


146    DISEASES   OF   THE   NERVOUS   SYSTEM 

digits,  but  also  innervating  four  muscles,  i.e.  the  abductor 
hallucis,  flexor  brevis  hallucis,  flexor  brevis  digitorum, 
and  the  1st  lumbrical.  The  external  plantar  supplies 
the  remaining  sensory  area  and  all  the  other  muscles  of 
the  foot.  As  the  digital  branches  from  the  plantar 
nerves  run  forwards  to  supply  the  skin  covering  the 
toes  they  pass  between  the  heads  of  the  metatarsal 
bones,  and,  as  a  result  of  the  crowding  together  of  the 
bones  by  pressure  of  tight  boots,  the  nerves  going  to 
supply  adjacent  sides  of  the  fourth  and  fifth  toes  may 
be  compressed.  Pain  may  not  only  be  felt  at  the  actual 
site  of  pressure,  but  may  radiate  up  nearly  as  high  as 
the  knee. 

SCIATICA 

Sciatica  is  one  of  the  most  common  of  the  nervous 
diseases. 

The  eaiises  may  be  divided  into  (1)  extrinsic,  such 
as  pressure  from  pelvic  tumours  and  spicules  of  bone, 
and  irritation  from  rheumatoid  changes  in  the  hip- joint, 
and  (2)  intrinsic,  depending  upon  inflammatory  processes 
in  the  nerve  trunk.  These  are  often  brought  about  by 
rheumatic  and  gouty  conditions,  and  in  some  cases  lead 
may  be  a  factor.  Injury,  strain,  damp,  and  cold  also 
appear  to  be  causes. 

Pathology. — An  interstitial  inflammation  of  the 
nerve  sheath  with  subsequent  injury  to  the  nerve  fibres 
is  the  most  common  lesion. 

Symptoms. — The  main  symptoms  are  pain  and 
tenderness.  The  pain  is  distributed  in  the  course  of  the 
nerve  in  the  thigh,  and  is  often  continued  down  the 
branches  of  the  nerve  beyond.  There  may  be  hyper- 
sesthesia,  and  in  a  few  cases  some  degree  of  anaesthesia. 
In  severe  cases,  tenderness  may  be  elicited  by  deep 
pressure  anywhere  along  the  line  of  the  nerve,  and  it  is 
nearly  always  present  at  certain  points,  e.g.  the  sciatic 
notch,  ih^  popliteal  space,  and  the  outer  side  of  the 
fibula,  at  which  places  the  nerve  is  more  readily  reached. 

The  pain  is  often  very  intense  and  may  prevent  the 
patient  from  sleeping.     The  position  of  greatest  ease  is 


SCIATICA  147 

obtained  by  flexing  the  leg  at  the  knee  and  hip,  and  any 
attempt  to  extend  the  leg  at  once  stretches  the  nerve 
and  causes  pain.  The  position  in  which  the  leg  is  kept 
causes  the  patient  to  stand  and  walk  in  such  a  manner 
that  the  weight  is  chiefly  thrown  on  to  the  opposite 
limb,  and  in  time  this  attitude  may  cause  some  lateral 
curvature  of  the  spine. 

In  severe  and  long-standing  cases  there  may  be 
atrophy  of  muscles,  but  in  the  recent  and  slighter  cases 
there  is  often  no  definite  loss  of  power.  Occasionally 
oedema  of  the  leg  and  herpes  are  present.  The  tendo- 
Achillis  jerk  is  sometimes  lost  on  the  affected  side  ; 
the  knee-jerk  generally  remains,  and  may  be  some- 
what exaggerated.  Lumbago  is  often  associated  with 
sciatica. 

Diagnosis. — The  diagnosis  of  sciatica  is  important, 
since  it  is  necessary  to  be  careful  not  to  overlook  pos- 
sible causes  of  pressure.  The  pelvis  must  be  examined 
for  signs  of  tumour,  and  the  spine  for  signs  of  tuber- 
culosis, with  which  a  psoas  abscess  may  be  associated. 
Account  must  also  be  taken  of  any  injury  or  disease 
of  the  pelvic  bones  and  hip-joint  (e.g.  rheumatoid  arth- 
ritis), the  effects  of  which  may  have  extended  to  the 
sciatic  nerve. 

The  lightning  pains  of  tabes  sometimes  settle  in  the 
course  of  the  sciatic  nerve  for  days  at  a  time.  Unless 
this  possibility  is  remembered,  their  nature  is  likely 
to  be  overlooked.  Suspicion  should  always  be  aroused 
if  the  "  sciatica  "  is  bilateral. 

Ti^eatment.  —  At  the  outset  of  an  acute  attack 
the  patient  should  be  kept  in  bed  and  given  a  purge. 
To  relieve  the  pain,  all  the  ordinary  forms  of  counter- 
irritation  can  be  tried,  and  of  these  heat  often  proves  the 
most  beneficial,  while  phenacetin,  aspirin,  and  drugs  of 
similar  nature  may  be  given  by  the  mouth.  In  severe 
cases,  drugs  may  be  administered  locally  by  cataphoresis. 
Where  the  pain  is  very  severe,  and  not  relieved  by  other 
measures,  morphia  may  be  required.  Applications  of  the 
galvanic  current  mayalso  help  to  relieve  the  pain. 


148    DISEASES   OF  THE  NERVOUS   SYSTEM 

As  soon  as  the  more  acute  symptoms  have  sub- 
sided, massage  and  electricity  may  often  be  prescribed 
with  success.  Massage  must  generally  be  light  at  first, 
though  occasionally  patients  obtain  considerable  after- 
benefit  from  kneading  in  the  acute  stages,  which  at 
the  time  gives  rise  to  a  good  deal  of  pain.  In  the  more 
chronic  cases,  applications  of  the  high-frequency  current 
are  often  useful. 

If,  after  the  acute  stage  of  the  disease  has  passed 
off,  no  improvement  takes  place,  the  question  of  nerve 
injections  may  be  considered.  In  the  case  of  the  sciatic 
nerve,  normal  saline  in  combination  with  some  novocain 
is  generally  used,  and  care  must  be  taken  not  to  inject 
alcohol  or  any  other  substance  that  will  lead  to  paralysis. 
Wilfred  Harris  recommends  that  the  nerve  be  first  in- 
jected near  the  great  sciatic  notch  with  2  c.c.  of  2-per- 
cent, novocain  solution,  and  then  at  the  same  point, 
immediately  afterwards,  with  70  to  100  c.c.  of  0*9-per- 
cent.  saline. 

Prognosis  varies.  Some  patients  recover  rapidly, 
while  in  others  the  disease  becomes  chronic  and  lasts 
for  weeks  or  months.  There  is  a  considerable  tendency 
to  recurrence. 

Local  Paralysis  following  Superficial  Injuries 
NOT  involving  Nerve  Trunks. 
Walshe*  has  recently  reviewed  an  interesting  group 
of  cases  characterized  by  signs  of  a  local  neuritis  follow- 
ing a  trivial  superficial  injury  to  the  distal  extremity 
of  a  hmb.  He  thinks  such  cases  for  the  most  part 
approximate  to  the  type  known  as  ascending  neuritis, 
rather  than  to  reflex  effects  such  as  may  follow  an  acute 
arthritis,  and  also  considers  that  a  small  proportion 
of  the  cases  described  by  Babinski  as  "  reflex  nervous 
disorders  "f  may  belong  to  the  same  class. 

*  Brain,  Vol.  xlii..  Pt.  iv.,  1919. 

t  During  tlio  war  Babinski  collected  a  numher  of  cases  which  he 
grouped  under  the  heading  of  "Troubles  Nerveux  d'Origine  Reflex," 
but  doubt  has  been  expressed  whetlier  the  group  was  not  made  uj)  of 
variou?  peculiar  abuornmlities  for  which  no  common  orighi  could  be 
assigned. 


LESIONS   OF   CAUDA  EQUINA  149 

The  Cauda  Equina 
In  lesions  of  the  cauda  equina  the  paralysis  is  of  the 
lower  neurone  type,  characterized  by  loss  of  power, 
wasting,  and  changes  in  the  electrical  reactions  of  the 
muscles  affected.  Control  over  the  sphincters  is  gener- 
ally lost,  as  also  is  sexual  power.  Loss  of  sensation  is 
extensive,  and  its  usual  distribution  over  the  buttocks 
in  a  "saddle- shaped"  area  is  especially  characteristic. 


CHAPTER   XIV 

MULTIPLE   NEURITIS 

Ix  multiple  or  polyneuritis  there  is  a  generalized  in 
flammation  of  some  or  all  of  the  fibres  forming  nerve 
trunks.  The  nerves  of  the  limbs  are  mainly  affected. 
The  symptoms  are  generally  symmetrical,  but  vary 
according  to  the  acuteness  of  the  onset  and  the  selective 
action  which  different  poisons  tend  to  exert. 

Selective  influence  of  poisons. — Many  poisons 
which  attack  the  nervous  system  have  an  apparent 
affinity  for  certain  neurones  before  they  produce  more 
generalized  symptoms,  and  a  knowledge  of  their  special 
tendencies  is  often  an  aid  to  diagnosis. 

Thus,  lead  shows  a  special  tendency  to  affect  the 
nerves  of  the  forearms,  alcohol  those  of  the  legs,  and 
diphtheria  those  of  the  soft  palate  and  eyes. 

Etiology. — Of  the  poisons  taken  directly  into  the 
system,  those  which  most  frequently  produce  neuritis 
are  alcohol,  lead,  and  arsenic,  and,  less  frequently,  copper 
and  mercury.  Toxins  which  are  the  direct  outcome  of 
bacterial  or  protozoal  infection  are  also  causes,  e.g.  those 
associated  with  diflitheria,  ty]}lwid,  malaria,  2Jneumonia, 
tiihercle,  and  other  infectious  diseases.  Of  these  the 
diplitlieria  toxin  is  by  far  the  most  imj^ortant,  and, 
indeed,  peripheral  neuritis  following  the  others  is  dis- 
tinctly uncommon.  The  toxin  of  acute  poliomyelitis 
also  sometimes  attacks  the  peripheral  nerves. 

The  toxins  associated  with  lejyrosy  and  beriberi 
are  potent  causes  in  countries  where  these  diseases 
are  prevalent. 

160 


MULTIPLE   NEURITIS  151 

Other  poisons,  probably  of  chemical  rather  than  of 
bacterial  origin,  which  may  cause  neuritis  are  those 
associated  with  gout,  rheumatism,  ancemia,  and  diabetes. 
Neuritis  may  also  follow  injuries  and  the  general 
enfeeblement  attending  wasting  diseases  and  senile 
degeneration. 

Pathology. — The  changes  found  in  the  nerves  in 
multiple  neuritis  are  those  of  a  parenchymatous  in- 
flammation and  degeneration,  to  which  may  be  added 
more  diffuse  inflammatory  changes  in  the  interstitial 
tissue  and  nerve  sheaths,  according  to  the  nature  and 
virulence  of  the  poison.  As  a  general  rule  the  changes 
are  most  advanced  in  the  terminal  filaments. 

In  many  instances  some  changes  have  been  observed 
in  the  motor  cells  of  the  cord  also,  thus  showing  that 
the  poison  has  a  tendency  to  attack  the  whole  of  the 
lower  motor  neurone. 

General  symptoiiiatology. — An  ordinary 
"  mixed ''  nerve  contains  motor  and  sensory  fibres, 
and  possibly  some  fibres  which  exercise  a  trophic 
influence,  although  the  independent  existence  of  these 
is  doubtful.  A  perijDheral  nerve  may  thus  be  divided 
into  its  various  component  fibres,  like  the  columns  of 
the  cord,  and,  as  in  the  latter,  all  the  i)Siits  need  not  be 
affected  equally. 

When  the  functions  of  all  the  fibres  are  impaired 
or  destroyed,  there  are  ^055  of  'poiver,  loss  of  sensation, 
and  loss  of  reflexes,  with  perhaps  some  trophic  changes 
in  the  skin,  which  last  are  not,  however,  of  so  acute  a 
nature  as  those  found  in  myelitis. 

The  loss  of  'power  is  mainly  in  the  limbs,  and  may  be 
confined  to  the  legs  or  to  the  arms,  or  may  be  present 
in  both.  The  weakness  is  usually  most  marked  in  the 
extensor  muscles  below  the  knees  and  elbows,  giving 
rise  to  "  dropped  foot  "  and  "  dropped  wrist."  The 
trunk  muscles  and  those  supplied  by  the  cranial  nerves 
are  more  seldom  affected,  but  the  pneumogastric  and 
phrenic  nerves  are  especially  liable  to  involvement  in 
postdiphtheritic  neuritis. 


152    DISEASES   OF   THE  NEKVOUS   SYSTEM 

The  signs  are  those  of  a  lower  neurone  lesion,  viz. 
^Ya sting  and  alterations  in  the  electrical  reactions  of 
muscles,  loss  of  reflexes,  and  a  tendency  for  the  limbs 
to  assume  abnormal  positions.  The  rapidity  with  which 
power  is  lost  varies  greatly,  and  in  some  of  the  acute 
toxsemic  cases  the  limbs  may  become  powerless  in  even 
a  few  hours.  The  knee-jerks  are  generally  lost  early, 
but  occasionally  they  are  temporarily  exaggerated 
during  the  period  of  nerve  irritation. 

Sensory  symptoms  are  often  among  the  earliest  to 
appear.  Apart  from  complete  anaesthesia,  irritation  of 
the  sensory  fibres  can  cause  every  variety  of  disturb- 
ance of  sensation,  including  pain,  tingling,  numbness, 
and  hyperaesthesia  of  different  intensities,  Wlien  definite 
anaesthesia  develops,  it  is  usually  symmetrical  in  the 
limbs,  and  corresponds  more  or  less  to  the  areas  over 
which  gloves  and  stockings  are  usually  worn,  and  hence 
it  has  received  the  name  of  "  glove-and-stccking  anaes- 
thesia." The  anaesthesia  does  not  extend  to  the  trunk. 
The  muscles  of  the  legs  are  often  very  tender,  especially 
in  the  alcoholic  cases,  and  in  some  instances  loss  of 
muscle  sense,  with  consequent  incoordination,  is  one  of 
the  most  prominent  symptoms.  The  intensity  and 
rapidity  of  the  onset  of  sensory  symptoms  are  very 
variable  ;  in  most  of  the  chronic  cases  the  subjective 
sensations  of  tingling,  numbness,  and  pins-and-needles 
come  on  gradually  and  are  the  first  symptoms  to 
which  attention  is  drawn.  The  extent  to  which  the 
different  forms  of  sensation  are  impaired  is  also  very 
variable  :  in  some  patients  all  forms  are  affected  ;  in 
others  the  loss  of  muscle  sense  may  be  disproportion- 
ately great,  with  a  resulting  ataxy  that  may  be  mis- 
taken for  that  of  tabes.  In  some  of  the  more  acute 
varieties  of  toxic  origin  sensations  may  be  but  little 
affected. 

Vaso-motor  and  trophic  symptoms  are  not,  as  a  rule, 
prominent.  (Edema  of  the  logs  may  be  occasion- 
ally observed.  The  "  trophic  "  changes  show  themselves 
chiefly  by  impaired  nutrition  of  the  skin,  which  becomes 


ALCOHOLIC   NEUElTLS  155 

smootli  and  glossy ;  of  tlie  nails,  which  become  brittle 
and  lined  ;  and  of  the  smaller  joints,  which  are  apt  to 
become  stifi  and  to  contract  adhesions.  Acute  bedsores 
do  not  occur,  and  control  over  the  sphincters  is  retained. 

Alcoholic  Neuritis 

Etiology. — Alcoholic  neuritis  occurs  from  exces- 
sive drinking  over  a  long  period  of  time.  Spirits  ap- 
pear to  be  the  more  potent  cause,  but  many  cases 
arise  from  drinking  beer.  Both  men  and  women  are 
aSected. 

Symptoms. — The  loss  of  power  nearly  always 
begins  in  the  feet  and  legs,  and  is  most  marked  below 
the  knees.  The  feet  drop  into  a  line  with  the  legs  {foot- 
drof),  and  if  the  patient  is  able  to  walk  the  gait  is  high- 
stepping  in  order  to  clear  the  toes  from  the  ground. 
The  arms  are  sometimes  affected,  and  palsies  of  cranial 
nerves  have  occasionally  been  observed.  The  wastifig  of 
the  paralysed  limbs  is  generally  very  pronounced,  and 
the  electrical  reactions  are  those  of  degeneration.  The 
knee-jerks,  tendo- AchUlis  jerks,  and  plantar  reflexes  are 
usually  lost. 

The  sensory  symptoms  are  prominent.  Thus,  pain 
is  often  excruciating,  and  the  muscles  of  the  calves 
may  be  very  tender  on  pressure,  although  the  latter 
sign  is  by  no  means  always  present.  Numbness  and 
tingling  are  common,  as  also  is  hypersesthesia.  The 
anaesthesia  takes  the  characteristic  ''  stocking-and- 
glove "  distribution  already  described.  The  muscle- 
sense  is  apt  to  be  greatly  diminished,  giving  rise  to 
ataxy  resembling  that  of  tabes. 

The  vision  occasionally  suffers  ;  there  may  be  am- 
blyopia or  impairment  of  colour  vision,  and  nystagmus 
and  optic  neuritis  have  both  been  known  to  occur, 
though  very  rarely. 

Mental  symptoms. — Emotional  tendencies,  untruthful- 
ness, indecision,  mental  confusion,  with  loss  of  memory 
for  recent  events  and  loss  of  ideas  of  space  and  time — • 
symptoms  which  are  grouped  under  the  designation  of 


154    DISEASES  OF  THE  NERVOUS  SYSTEM 

Korsakow^s  psychosis — are  generally  met  with  in  some 
degree,  while  in  the  more  severe  cases  hallucinations, 
delirium  tremens,  or  insanity  may  be  present.  These 
mental  changes  are  sometimes  associated  with  loss 
of  control  over  the  sphincters  —  a  symptom  which 
is  not  present  in  uncomplicated  cases  of  multiple 
neuritis. 

Pathology. — The  chief  changes  are  parenchymatous 
inflammation  and  degeneration  of  nerve  fibres,  the  ter- 
minal twigs  suffering  most  severely.  The  cells  of  the 
cord  and  cortex  may  also  be  altered,  and  it  is  to  changes 
in  the  latter  that  the  cerebral  symptoms  are  due. 

Arsenical  Neuritis 

Etiolociy. — Arsenical  poisoning  has  occurred  from 
the  inhalation  of  particles  arising  from  various  decora- 
tive articles  into  the  composition  of  which  arsenic  has 
entered,  as  green  wall-papers  and  carpets.  Arsenic  may 
also  accidentally  contaminate  articles  of  diet  in  sufficient 
quantities  to  cause  symptoms,  as  occurred  in  an  epidemic 
among  beer-drinkers  due  to  the  presence  of  arsenic  in 
glucose.  Neuritis  may  also  be  caused  by  long-continued 
use  of  arsenic  as  a  medicine. 

Symptoms. — There  is  a  close  similarity  between 
the  symptoms  of  arsenical  neuritis  and  those  of  alcoholic 
neuritis,  to  which  the  reader  is  referred. 

The  legs  are  generally  affected  first  (Fig.  33),  and 
pain,  tingling,  and  numbness  are  accompanied  by  weak- 
ness of  the  extensors  and  by  ataxy.  Later  on,  the 
paralysis  may  appear  in  the  arms. 

Arsenical  neuritis  is  accompanied  by  pigmentation 
of  the  skin,  which  is  a  symptom  of  diagnostic  import- 
ance. The  skin  assumes  a  deep-brownish  colour,  which 
is  most  apparent  on  the  trunk  and  extensor  surfaces 
of  the  limbs.  Herpes,  keratosis  of  the  palms  of  the 
hands  and  soles  of  the  feet,  erythema,  erythromelalgia, 
changes  in  the  structure  of  the  nails,  and  oedema  of  the 
extremities  may  all  be  present.  Recovery  generally 
takes  place  very  slowly. 


NEURITIS  FROM  LEAD  155 

Lead  Neuritis 
Etiology. — This  form  of  neuritis  is  chiefly  found  in 
painters,  who  inhale  the  poison,  and,  unless  they  exer- 
cise great  care,  also  contaminate  their  food  with  par- 
ticles that  have  clung  round  the  finger-nails.  Workers 
with  metallic  lead,  such  as  type-setters,  are  also  liable 
to  the  disease.  Another  source  of  poisoning  is  the  con- 
tamination of  water  or  other  drink  with  lead. 


Fig.  33.— "Foot-drop"  in  arsenical  neuritis.     (By  courtesy  of 
Br.  George  Ogilvie.) 

Symptoms. — Neuritis  does  not  usually  occur  until 
the  patient  has  been  exposed  to  the  influence  of  lead 
for  a  long  time,  and  it  is  generally  preceded  by  other 
indications  of  lead  poisoning,  such  as  colic,  dyspepsia, 
constipation,  and  anaemia. 

The  characteristic  blue  line  on  the  gums  can  also 
often  be  observed,  and  analysis  of  the  urine  and  faeces 
may  afiord  confirmatory  evidence  of  the  presence  of 
lead  in  the  body.  The  inflammatory  and  degenerative 
changes  are  chiefly  found  in  the  peripheral  nerves,  but 
lesions  can  also  occasionally  be  demonstrated  in  the 
motor  cells  of  the  cord. 


156    DISEASES   OP   THE  NERVOUS   SYSTEM 

The  extensor  muscles  of  the  forearms  and  fingers  are 
weakened,  causing  wrist-drof  and  inability  to  straighten 
the  fingers  (Fig.  34). 

The  supinator  longus  escapes,  the  paralysis  being 
usually  limited  to  the  distribution  of  the  posterior  inter- 
osseous branch  of  the  musculo-spiral  nerve.  Though 
the  flexors  of  the  forearm  are  not  paralysed,  the  grasp 
is  greatly  weakened  owing  to  the  inability  of  the  ex- 
tensors to  fix  the  wrist.     One  hand  is   often  affected 


Fig.  3J:.— Wrist-drop  in  neuritis  from  chronic  lead-poisoning. 

before  the  other,  and  to  a  greater  degree.  After  the 
dropped-wrist  condition  has  existed  for  some  time,  a 
swelling  appears  on  the  back  of  the  hand,  due  partly 
to  displacement  of  the  bones  of  the  wrist  and  partly  to 
changes  in  the  synovial  membranes.  There  is  seldom 
any  definite  disturbance  of  sensation.  Another  variety, 
which  may  occur  independently  of.  or  in  association 
with,  the  forearm  distribution,  is  that  in  which  the  loss 
of  power  affects  the  small  muscles  of  the  hand.      The 


POSTDIPHTHERITIC    NEURITIS  157 

interossei  and  muscles  of  the  thenar  eminence  waste 
and  the  signs  closely  resemble  those  of  progressive 
muscular  atrophy.  Only  in  severe  cases  does  the  para- 
lysis extend  beyond  the  forearms.  In  rare  instances  a 
spasm  of  the  muscles  occurs.  In  some  cases  there  are 
symptoms  of  disturbance  of  the  central  nervous  system, 
such  as  headache,  vomiting,  mental  impairment,  and 
optic  neuritis ;  paralysis  of  cranial  nerves  has  occa- 
sionally been  observed.  Recovery  generally  takes  place 
very  slowly,  and  the  patient  is  liable  to  relapse  if  he 
again  comes  under  the  influence  of  the  poison. 

Postdiphtheritic  Neuritis 

Postdiphtheritic  paralysis  occurs  in  children  and 
adults,  but  more  often  in  the  former.  The  first  signs 
may  show  themselves  a  few  days  after  the  disease  has 
commenced,  or,  on  the  other  hand,  they  may  not  appear 
for  several  weeks  ;  the  usual  period  of  onset  is  about  a 
fortnight  after  infection. 

Symptoms. — In  the  majority  of  cases  paralysis 
of  the  soft  palate  is  the  first  symptom,  as  evidenced 
by  the  regurgitation  of  fluids  through  the  nose,  and  the 
presence  of  a  "  nasal  "  voice. 

The  ocular  group  of  muscles  is  generally  the  next 
to  be  affected,  and  though  any  of  these  may  be  paralysed, 
the  external  rectus  and  the  cihary  muscles  are  those 
most  frequently  weakened.  In  severe  cases  the  para- 
lysis is  more  generalized,  and  there  may  be  loss  of  power 
in  all  the  hmbs. 

Of  particular  importance  is  the  vulnerabihty  of  the 
vagus  and  the  phrenic  nerves  to  the  effects  of  the  toxin. 

The  knee-jerks  are  generally  lost  early,  and  indeed 
they  are  often  absent  after  an  attack  of  diphtheria 
without  any  other  symptoms  of  neuritis.  There  may 
be  ataxy  of  the  limbs,  and  sensory  symptoms,  such  as 
pain,  tingling,  and  anaesthesia,  may  be  complained  of. 
Vomiting  is  sometimes  a  troublesome  symptom. 

Observations  made  by  Walshe*  show  that  an  initial 
*  Froc.  Boy.  Soc\  Med.y  March,  1920, 


158    DISEASES   OF   THE  NERVOUS  SYSTEM 

local  paresis  frequently  occurs  at  the  site  of  the  infective 
focus,  wherever  the  latter  may  be,  e.g.  an  infection  of  the 
thumb  gave  rise  to  a  neuritis  of  the  arm  independent 
of  the  generalized  multiple  neuritis  which  followed. 
Walshe  considers  that  the  palatal  paralysis  in  faucial 
diphtheria  is  a  corresponding  local  manifestation  at  the 
site  of  infection,  so  that  the  complete  symptom-complex 
in  any  given  case  would  consist  of  (1)  an  initial  local 
paresis  related  anatomically  to  the  site  of  the  infective 
focus,  (2)  paralysis  of  accommodation,  (3)  polyneuritis. 

Course  and  prognosis. — Many  cases  run  an  un- 
eventful course,  and  power  gradually  returns.  When 
the  paralysis  does  not  spread  beyond  the  soft  palate 
and  the  ocular  muscles,  recovery  often  takes  place 
quickly,  but  when  the  limbs  are  affected  the  duration 
is  much  longer,  and  it  may  be  several  months  before 
power  is  restored.  Signs  that  the  power  of  the  heart 
or  of  the  diaphragm  is  impaired  always  give  the  case 
a  grave  aspect.  "  Bulbar  crises "  characterized  by 
failure  of  heart  and  respiration  may  occur,  and  these 
are  often  associated  with  paralysis  of  the  diaphragm 
(Pasteur). 

Pathology, — The  toxin  appears  to  affect  the  whole 
of  the  lower  motor  neurone,  and  a  parenchymatous 
degeneration  of  the  myelin  sheath  of  the  nerves  seems 
to  be  the  most  important  lesion  found  after  death  (F.  E. 
Batten). 

Changes  in  the  motor  nuclei  of  the  cord  and  bulb 
have,  however,  also  been  described,  and  alterations  in 
the  structure  of  the  motor  cells  of  the  anterior  horns 
of  the  cord  have  been  noted  in  animals  at  quite  an  early 
stage  of  dijjhtheria  intoxication.*  Walshe  considers  it 
probable  that  the  initial  local  ])alsy  is  due  to  the  passage 
of  the  toxin  along  the  perineural  lymph-channels  of  the 
peri])heral  nerves  {see  p.  74),  the  multiple  neuritis  being 
the  result  of  a  generalized  blood  infection. 

Fatty  degeneration  has  been  observed  in  the  muscle 

*  A.  G.  R.  Foulerton  and  Campbell  Thomson,  7iV/e».  Med.  Jonrn.y 
J*n,,  1902, 


MULTIPLE    NEURITLS:    DIAGNOSIS       159 

tissue  of  the  heart  and  diaphragm,  and  also  in  other 
viscera,  and  is  regarded  by  Dudgeon  as  the  essential 
cause  of  the  acute  cardiac  failure  which  is  apt  to  occur 
in  these  cases. 

A  type  known  as  acute  febrile  polyneuritis 
sometimes  occurs.  Its  characteristic  features,  as 
described  by  Gordon  Holmes,*  are  those  of  a  rapidly 
developing  widespread  flaccid  paralysis  which  often 
affects  the  muscles  of  the  face  and  deglutition,  with 
occasional  involvement  of  the  muscles  of  the  eyes 
and  sphincters.  Sensory  changes  are  usually  slight. 
This  variety  has  to  be  differentiated  from  neuritis 
due  to  other  causes,  and  from  cord  lesions,  e.g. 
acute  ascending  myelitis,  poliomyelitis,  and  Landry's 
paralysis. 

Diagnosis  of  multiple  neuritis.  —  Peripheral 
neuritis  has  to  be  distinguished  from  the  various  forms 
of  myelitis.  The  main  distinguishing  features  are  the 
signs  in  neuritis  of  a  lower  neurone  lesion,  and  the 
absence  of  sphincter  trouble.  A  transverse  myelitis 
above  the  lumbar  enlargement  is  associated  with  the 
signs  of  an  upper  neurone  lesion  together  with  loss  of 
control  over  the  sphincters. 

Other  important  differences  are  to  be  found  in  the 
distribution  of  the  anaesthesia  and  in  the  formation  of 
bedsores. 

In  transverse  myelitis  there  is  anaesthesia  below  the 
level  of  the  lesion,  but  in  neuritis  the  sensory  loss  is 
confined  to  the  limbs  and  does  not  extend  to  the  trunk. 
The  acute  bedsores  that  are  so  commonly  associated 
with  myelitis  do  not  occur  in  neuritis. 

A  myelitis  of  the  lumbar  enlargement  may  super- 
ficially resemble  a  peripheral  neuritis,  inasmuch  as  the 
knee-jerks  may  be  lost,  and  the  muscles  of  the  thighs 
and  legs  may  waste  from  destruction  of  the  motor 
cells  in  the  cord  at  the  level  of  the  lesion,  but  the 
complete  loss  of  control  over  the  sphincters  which 
*  Brit,  Med,  Journ.,  June  14,  1917. 


160    DISEASES   OF   THE   NERVOUS   SYSTEM 

occurs  in  these  circumstances  is  still  a  distinguishing 
feature. 

From  acute  anterior  poliomyelitis  the  chief  distinc- 
tions are  the  alterations  of  sensation  and  the  symmetry 
of  the  paralysis  as  compared  with  the  irregularly  scat- 
tered lesions  of  poliomyelitis.  There  are,  however, 
some  cases  in  Tvhich  it  is  very  difficult  to  arrive  at  a 
differentiation. 

From  Landry'^s  "paralysis  there  is  also  a  difficulty* 
in  some  cases,  in  distinguishing  peripheral  neuritis, 
though  in  characteristic  examples  of  Landry's  disease 
the  absence  of  any  alteration  of  sensation,  of  wasting, 
and  of  electrical  changes  in  the  muscles  is  a  distin- 
guishing feature. 

The  symptoms  in  certain  cases  of  neuritis  closely 
simulate  those  of  locomotor  ataxy.  The  resemblance 
occurs  when  incoordination  is  a  prominent  feature, 
and,  where  this  exists  in  combination  with  absent 
knee-jerks  and  the  presence  of  pains  in  the  legs,  tabes 
is  apt  to  be  diagnosed.  Some  cases  of  alcoholic  neuritis 
closely  resemble  tabes  in  these  particulars,  as  also 
do  some  cases  of  neuritis  due  to  diabetes,  in  which 
the  similarity  may  be  marked  by  the  presence  of  gan- 
grenous ulcers  on  the  feet,  but  the  Argyll-Robertson 
pupil  of  tabes  and  the  loss  of  power  with  the  altered 
electrical  reactions  in  neuritis  will  assist  the  diagnosis. 

From  hysterical  paralysis  neuritis  can  be  differen- 
tiated by  the  absence  in  the  latter  of  knee-jerks  and 
the  presence  of  wasting  and  changes  in  the  electrical 
reactions  of  the  muscles. 

Prognosis. — The  prognosis  varies  with  the  nature 
of  the  cause  and  the  possibility  of  its  removal.  In 
general  the  chances  of  recovery  of  power  are  good  if 
the  cause  can  be  removed,  but  the  progress  is  often 
very  slow. 

In  alcoholic  neuritis,  when  the  cause  is  removed 
improvement  usually  takes  place,  except  in  the  most 
severe  cases,  in  which  death  may  occur  from  general 
exhaustion  or  cardiac  failure. 


MULTIPLE   NEURITIS  :  TKEATMENT       161 

Signs  of  impairment  of  the  functions  of  the  vagus 
or  phrenic  nerves  are  always  grave,  and  the  special 
liability  of  these  to  be  damaged  in  postdiphtheritic 
paralysis  has  been  already  mentioned. 

Treatment. — It  is  obvious  that,  whenever  possible, 
the  cause  must  be  removed  at  once. 

Little  can  usually  be  done  to  shorten  the  process  of 
recovery,  but  salicylate  of  soda  and  iodide  of  potassium 
sometimes  appear  to  have  favourable  effects.  After 
the  acute  symptoms  have  subsided,  strychnine  is 
probably  the  most  useful  drug.  When  the  pains  are 
severe,  local  applications  of  heat  and  liniments  con- 
taining chloroform  and  belladonna  are  useful,  and  occa- 
sionally alternate  successive  applications  of  heat  and 
cold  give  relief.  Phenacetin,  antipyrin,  antifebrin,  aspi- 
rin, and  salicylates  are  the  drugs  that  are  most  useful 
as  anodynes.  In  the  worst  cases  recourse  must  be  had 
to  morphia. 

Massage  and  electrical  treatment  should  be  com- 
menced early,  and  care  must  be  taken  to  prevent  the 
paralysed  limbs  from  assuming  faulty  positions,  espe- 
cially with  regard  to  the  feet. 

In  the  postdiphtheritic  cases  it  is  most  important 
to  keep  the  patient  at  rest  till  the  danger  of  cardiac 
failure  is  over,  for  fatal  syncope  may  result  from  the 
patient  merely  sitting  up  in  bed.  During  the  attacks  of 
respiratory  failure  which  are  apt  to  occur  in  these  cases, 
artificial  respiration  may  tide  over  the  time  of  danger. 

When  lead  has  caused  the  neuritis,  its  elimination 
should  be  hastened  by  the  regular  administration  of 
saline  purgatives,  of  which  magnesium  sulphate  is  the 
one  most  often  chosen.  Iodide  of  potassium  is  also  sup- 
posed to  assist  the  elimination  of  both  lead  and  arsenic, 
but  the  results  of  analyses  of  the  excreta  seem  to  show 
that  it  has  not  much  influence,  at  any  rate  so  far  as  the 
elimination  of  lead  is  concerned. 


CHAPTER   XY 

TUMOURS    OF    NERVES    (NEUROMATA) 

Nerve  trunks  are  sometimes  the  seat  of  tumours 
which  are  usually  all  grouped  under  the  clinical  title 
of  neuromata,  though  their  structure  varies  and  is 
indeed  seldom  that  of  nerve  tissue.  Those  most  fre- 
quently met  with  are  neui^o-fibroniata,  which  form 
fusiform  swellings  on  the  nerve  trunks,  and  are  very 
liable  to  undergo  myxomatous  degeneration.  They 
mav  grow  from  the  cranial  or  the  spinal  nerves,  and 
are  often  multiple. 

Plexilorm  neui'oniata  (Fig.  35)  are  rare.  They 
form  more  diffuse  tumours  than  the  neuro-fibromata 
and  feel  "  like  a  bag  containing  a  number  of  tortuous, 
irregular  vermiform  bodies,  soft  to  the  touch  and 
mobile"   (Bland-Sutton). 

Closer  examination  in  these  cases  shows  that  multiple 
connective-tissue  tumours  have  formed  along  the  course 
of  the  nerves.  Of  the  cranial  nerves  the  trigeminal  is 
the  most  frequently  attacked,  but  the  gro^^i^h  may 
occur  in  any  nerve,  cranial  or  spinal. 

Ganglionic  neui»omata  are  very  rarely  met 
with.  They  are  formed  of  nerve  cells,  nerve  fibres, 
and  neuroglia: 

Neuro-fibromata  may  arise  in  great  numbers  from 
the  twigs  of  the  cutaneous  nerves  and  constitute  the 
condition  known  as  molluscum  fibrosum,  which,  when 
associated  with  freckles  or  a  more  diffuse  pigmenta- 
tion of  the  skin,  is  known  by  the  name  of  von  Reckling- 
hausen's disease; 

162 


TUMOUES  OF  NERVES:  SYMPTOMS  163 


Symptoms. — The  symp- 
toms depend  largely  on  where 
the  tumours  happen  to  be 
situated.  Growths  on  sensory 
nerves  may  cause  pain  and 
various  disturbances  of  sensa- 
tion ;  among  the  most  painful 
are  those  which  arise  on  the 


Fig.  36.  —  Cervical  segment  of  the 
cord  represented  in  Fig.  3.j.  A 
nodule  on  one  of  the  cervical 
roots  compressed  the  cord  and 
led  to  fatal  paraplegia.  (Sir  J. 
Bland- Sutton.) 


nerve-endings  of  an  amputa- 
tion stump. 

Ptessure  on  spinal  roots 
may  cause  w^eakness  in  the 
corresponding  muscles,  which 
may  be  followed  later  by 
signs  of  paraplegia  as  the 
tumour  invades  the  spinal 
cord  (Fig.  36). 

Prognosis.  —  Tumours 
growing   from    nerves    are 


Fig.  35.— Plexiform  neuromata  affect- 
ing the  roots  of  the  cauda  equina 
and  anterior  crural  nerve.  (Sir  J. 

Bland-Sutton.) 


164    DISEASES   OF   THE   NERVOUS   SYSTEM 

nearly  always  simple,  and  if  removed  show  no  tendency 
to  recur. 

There  is,  however,  some  danger  of  sarcomatous  de- 
velopment in  the  case  of  molluscum  fibrosum. 

Death  may  be  caused  by  pressure  on  the  cord  or 
brain. 

Treatment. — When  the  tumour  is  accessible  and 
causing  unpleasant  symptoms  it  should  be  removed,  but 
when  the  growths  are  multiple  the  difficulties  are  naturally 
greater,  and  in  the  molluscum  fibrosum  variety  nothing 
can  be  done. 


SECTION    III.-MUSCULAR 
DYSTROPHIES 

CHAPTER  XVI 

THE   MYOPATHIES;    AMYOTONIA    CONGENITA; 
PERONEAL  TYPE   OF   ATROPHY 

THE   MYOPATHIES 

Under  tlie  term  "  myopatliies  "  is  comprised  an  im- 
portant group  of  cases  in  which  the  failure  of  vitality 
appears  to  arise  in  the  muscles  themselves.  The  essential 
feature  of  these  cases  is  atrophy  of  the  muscles,  with 
which,  however,  there  may  be  some  accompanying  en- 
largement. In  many  of  the  forms  there  are  no  definite 
changes  to  be  found  in  the  nervous  system,  but  in  some 
varieties,  e.g.  the  peroneal  type,  a  neuronic  degeneration 
is  associated  with  the  atrophy. 

Morbid  auatom y. — ^Atrophy  of  some  fibres, 
pseudo-hypertrophy  of  others,  and  excess  of  fat  and 
fibrous  tissue  are  the  principal  changes  observed  in 
the  affected  muscles.  While  all  these  cases  seem  to  be 
pathologically  similar  in  their  want  of  vitality,  they 
vary  considerably  in  their  clinical  appearances.  Among 
the  forms  most  commonly  met  with  are  : 

1.  The  pseudo -hypertrophic  form. 

2.  The  facio-scapulo-humeral  form,  known  as  the 

Landouzy-Dejerine  type. 

3.  The  juvenile  form  of  Erb. 

There  have  been  described  also  a  "  distal "  form,  in 
which  the  atrophy  begins  in  the  extremities  of  the 
limbs,  and  a  "  myotonic  "  form,  in  which  atrophy  is 
associated  with  tonic  contractions  of  the  muscles  that 
relax   slowly   after  voluntary  movements.     Amyotonia 

165 


166    DISEASES  OF   THE  NERVOUS   SYSTEM 

congenita  and  the  peroneal  type  of  atropliy  are  also 
thought  to  be  closely  allied  to  this  group. 

As  might  be  expected  from  their  common  patho- 
logical basis,  no  sharp  line  can  be  drawn  between  the 
different  forms.  Cases  in  which  atrophy  is  the  dominant 
feature  often  show  traces  of  pseudo-hypertrophy,  while 
cases  of  pseudo  -  hypertrophy  almost  invariably  show 
some  signs  of  atrophy. 

Like  the  parallel  cases  of  spinal  origin,  the  myopathies 
are  very  liable  to  occur  in  several  members  of  a  family. 

1.  Pseudo-Hypertrophic  Paralysis 

Etiology. — This  disease  generally  begins  between  the 
ages  of  4  and  10,  It  exhibits  a  distinct  tendency  to 
run  in  families,  and  is  usually  transmitted  by  females 
and  inherited  by  males. 

Symptoms. — Clumsiness,  frequent  falls,  and  a  diffi- 
culty in  going  upstairs  are  generally  among  the  early 
symptoms,  all  of  which  seem  the  more  strange  to  the 
parents  since  the  limbs,  especially  the  calves,  often 
appear  to  them  to  be  unusually  well  developed.  Examina- 
tion, however,  shows  that  the  legs  are  weak  in  spite  of 
their  increased  size,  the  latter  proving  to  be  a  pseudo- 
hypertrophy due  to  overgrowth  of  fat  and  connective 
tissue  which  have  displaced  the  wasted  muscle  fibres. 

Distribution  of  hyjpertro'phy . — The  muscles  in  which 
some  enlargement  can  most  usually  be  seen  are  those 
of  the  calf  (Fig.  37),  the  quadriceps  extensor,  the  glutei, 
the  triceps,  and  the  infraspinati.  They  are  hard  and 
firm  to  the  touch,  but  despite  this  they  are  weaker  than 
they  should  be.  In  some  cases  part  of  a  muscle  is 
enlarged  and  part  atrophied. 

Muscles  most  commonly  atrophied. — Some  muscles 
are  from  the  first  atrophied  and  show  no  sign  of  enlarge- 
ment ;  among  them  the  most  constant  are  the  lower 
half  of  the  pectoralis  major  and  the  latissimus  dorsi. 
Others  in  which  atrophy  can  very  frequently  be  detected 
are  the  biceps,  the  flexors  of  the  knee,  and  the  pcronei 
(Fig.  37). 


PSEUDO-HYPERTROPHIC   PARALYSIS     167 

Certain  muscles  generally  escape,  e.g.  the  muscles 
of  the  face,  forearms,  and  hands,  but  occasionally  some 
enlargement  of  the  tongue  may  be  observed. 

Attitude. — The  failure  of  muscular  power  makes 
it  difficult  for  the  patient  to  stand,  walk,  or  rise  from 
the    ground,   and,   in   endeavouring   to    perform  these 


Fig.  37.— Case  of  pseudo-hyper- 
trophic  paralysis.  {Pierre 
Marie.)  Observe  the  en- 
largement of  the  muscles 
of  the  thighs  and  calves 
as  compared  with  the  thin 
arms. 


Fig.  38.— Deformities  arising 
in  the  course  of  pseudo- 
hypertrophic paralysis. 
{Pierre  Marie.)  This  is 
the  brother  of  the  patient 
in  Fig.  37. 


actions,  fairly  characteristic  attitudes  are  assumed.  In 
standing  the  feet  are  kept  wide  apart  in  order  to  obtain 
a  broader  base,  while  the  shoulders  are  thrown  back 
(lordosis)  in  order  to  keep  the  line  of  gravity  from  falling 
too  far  forwards.  The  arms  are  generally  flexed  and 
adducted. 


168    DISEASES   OF  THE  NERVOUS   SYSTEM 

In  walking  the  same  position  is  preserved,  and  the 
patient  waddles  from  side  to  side,  with  the  feet  turned 
out,  and  with  a  "  high -stepping  "  gait.     (Plate  7.) 

In  rising  from  the  supine  position  the  patient  first 
rolls  over,  then  takes  his  fixed  points  from  his  hands  and 
toes,  and  finally  helps  himself  up  by  placing  his  hands 
on  his  thighs. 

All  these  positions  are  due  to  attempts  to  compensate 
for  the  weakness  of  the  affected  muscles,  and  therefore 
they  vary  widely  in  degree  in  different  cases. 

Reflexes. — Wasting  of  muscles  leads  to  the  loss  of 
any  reflexes  which  they  may  normally  serve  ;  thus  the 
knee-jerk  diminishes  as  changes  occur  in  the  quadriceps 
extensor  muscle. 

Deformities. — Talipes  of  the  feet  and  curvature  of  the 
spine  are  both  often  met  with.     (Figs.  38,  42.) 

2.  Facio-Scapulo-Humkral  Type 

In  the  facio-scapulo-humeral  type  (known  also  as  the 
Landouzy-Dejerine  form)  the  atrophy,  like  that  of 
pseudo-hypertrophic  paralysis,  generally  begins  in  child- 
hood. It  is  often  spoken  of  as  the  "  infantile  form  "  of 
myopathy  in  contradistinction  to  the  "  juvenile  form," 
which  begins  in  later  fife. 

The  name  "  facio-scapulo-humeral "  shows  the  chief 
distribution  of  the  wasting.     (Fig.  39.) 

In  the  face  the  orbicularis  palpebrarum  and  the 
orbicularis  oris  are  commonly  weakened,  and  as  a  result 
of  the  weakness  of  the  latter  muscle,  together  with 
others  at  the  corner  of  the  mouth,  quite  a  characteristic 
expression  is  given  to  the  features. 

Next,  the  muscles  of  the  shoulder-girdle  and  upper 
arm  atrophy,  and  here  the  trapezius  (both  upper  and 
lower  divisions),  latissimus  dorsi,  serratus  magnus,  pec- 
torals, triceps,  and  biceps  generally  suffer  early.  The 
wasting  of  the  serratus  magnus  causes  the  vertebral 
border  of  the  scapula  to  tilt  whenever  the  arm  is 
lifted. 

The  forearms  generally  escape,  the  hands  always. 


PLATE    7.  —  Instantaneous   photograph    showing    "high- 
stepping'  gait  in  psendo-hypertrophic  paralysis. 
{By  courtesy  of  Br.  George  Ogilvie.) 


LANDOUZY-DEJERINE   PARALYSIS       169 

From  the  shoulder-girdle  the  wasting  spreads  to  other 
muscles  of  the  trunk  and  limbs.  The  muscles  of  the 
back  and  hip  become  weak,  causing  lordosis  and  a 
waddling  gait  like  that  in  the  pseudo-hypertrophic 
form,  while  wasting  of  the  other  muscles  is  accompanied 
by  difficulty  in   walking   and   deformities   of  the   feet. 


Fig.  39.— Appearance  of  neck  produced  by 
displacement  of  the  scapulae  and  drop- 
ping of  the  shoulders  in  a  case  of 
myopathy.    {Pierre  Marie.) 

Some  muscles  or  parts  of  muscles  are  often  found  to  be 
enlarged  (Fig.  40),  and  thus  a  relationship  is  established 
between  this  and  the  pseudo-hypertrophic  form. 

3.  Erb's  Juvenile  Form 
In  the  juvenile  form  the  wasting  is  generally  noticed 
rather    later   in    life,    but    nearly   always    before    the 
patient   is  out   of   his   teens.     The   wasting   generally 


170    DISEASES   OP  THE  NERVOUS  SYSTEM 

begins  about  the  shoulders  and  then  spreads  to  the 
trunk  (Fig.  41)  and  legs  ;  the  muscles  of  the  face  are 
also  sometimes  affected.  Enlargement  of  some  muscles 
may  be  observed. 

Diarjnosis  of  the  myopathics. — From  progressive 
muscular  atrophy  of  spinal  origin  the  chief  points  of  dis- 
tinction are  : 

i.  The  age  at  which  the  disease  begins. 


Fig.  10.— Case  of  myopathy 
showing  enlargement 
of  the  infraspinati. 
(Pierre  Marie.) 


ii.  The  distribution  of  the  wasting.  In  the  myo- 
pathies now  under  consideration  the  muscles  of  the 
trunk  waste  early,  the  hands  usually  escape,  and  the 
deltoid  is  often  normal ;  whereas  in  progressive  mus- 
cular atrophy  of  spinal  origin  the  hands  and  also  the 


PLATE  8.  — Case  of  myopathy  show- 
ing the  characteristic  attitude  of 
lordosis. 


MYOPATHIES:    DIAGNOSIS  171 

deltoid  are  among  the  earliest  parts  to  exhibit  signs  of 
the  disease. 

iii.  The  myopathies  tend  to  occur  in  several  members 
of  a  family,  and  when  this  peculiarity  is  present  the 
spinal  variety  may  be  excluded. 

iv.  Hypertrophy,  either  true  or  false,  is  not  found 
in  the  cases  of  spinal  origin. 

V.  Fibrillary  tremors  are  common  in  the  spinal  cases 
and  rare  in  the  myopathies.- 


Pig.  41.  —  Case  of  myopathy  showing 
wasting  of  the  muscles  of  the  trunk 
causing  the  deformity  of  the  trunk 
and  the  waist  known  as  the  "  taille 
cle  Quepe."    {Pierre  Marie.) 

The  distribution  of  the  wasting,  absence  of  sensory 
changes,  and  character  of  the  electrical  reactions  serve 
to  differentiate  these  cases  from  peripheral  neuritis. 

The  electrical  changes  shown  in  the  myopathies  are 
usuallv  those  of  diminished  reactions  to  both  the  faradic 


172    DISEASES   OF  THE   NERVOUS  SYSTEM 

and  galvanic  currents.  There  is  generally  no  definite 
"  reaction  of  degeneration." 

Prognosis. — ^All  these  different  forms  of  the  myo- 
pathies tend  to  progress  slowly,  but  here  and  there  arrest 
takes  place  or  the  rate  of  progression  is  so  slow  that  it 
can  be  ignored. 

The  danger  to  life  Hes  in  the  atrophy  extending  to 


Fig.  42,— Curvature  of  the  spiue  occurring 
in  the  course  of  myopathy.  (Pierre 
Marie.) 

the  muscles  of  respiration,  and  consequently  in  the 
failure  to  combat  successfully  any  pulmonary  complica- 
tions such  as  bronchitis,  pneumonia,  and  tubercle. 

Treatment. — Every  effort  to  arrest  the  wasting 
must  be  made.  Massage,  moderate  exercise,  and  elec- 
tricity should  all  be  used  to  keep  up  the  nutrition  of  the 
muscles,  and  must  be  persevered  with  if  any  success 
is  to  be  obtained. 


MUSCULAK   DYSTROPHIES  173 

AMYOTONIA   CONGENITA    (MYATONIA 
CONGENITA) 

In  contradistinction  to  the  condition  known  as  myo- 
tonia congenita,  described  at  p.  472,  there  is  an  un- 
common condition  of  obscure  origin,  which  was  first 
described  by  Oppenheim,  and  to  which  the  title  of 
amyotonia  (or  myatonia)  congenita  has  been  given. 
The  condition  appears  to  be  associated  with  myopathy 
in  some  cases,  while  in  others  the  connection  is  not  so 
clear.  It  also  occurs  in  the  atonic  form  of  cerebral 
diplegia  of  children. 

Etiology. — The  disease  is  met  with  in  children, 
and  in  the  majority  of  cases  has  either  existed  from 
the  time  of  birth  or  come  on  very  shortly  afterwards. 
In  a  few  the  history  gives  rise  to  suspicions  that  the 
trouble  has  followed  some  illness,  e.g.  measles  in  early 
childhood,  but  it  is  possible  that  the  condition  has 
pre-existed,  and  only  become  more  obvious  through 
the  illness. 

Symptoms. — The  characteristic  features  in  these 
cases  are  weakness  and  absence  of  muscle  tone,  so  that 
the  limbs  can  be  placed  in  all  kinds  of  grotesque  atti- 
tudes.    (Figs.  43,  U.) 

The  distribution  of  the  disability  varies  in  differ- 
ent cases.  In  some  cases  the  face,  trunk,  and  limbs 
may  all  be  affected,  but  in  others  the  muscles  of  the 
limbs,  and  especially  of  the  legs,  are  the  only  parts 
which  lose  their  tone. 

The  power  of  movement  is  greatly  diminished,  so 
that  the  child  cannot  stand  or  walk,  and  when  the 
trunk  muscles  are  toneless  it  may  be  unable  even  to  sit 
up.  If  the  face  is  affected  there  may  be  inability  to 
close  the  eyes. 

The  muscles  in  the  diseased  areas  are  flabby,  and  a 
faradic  current  of  unusual  strength  is  necessary  to 
make  them  contract ;  moreover,  currents  of  far  greater 
intensity  than  can  be  borne  by  a  normal  child  appear 
to  cause  no  pain. 


174    DISEASES   OF   THE   NERVOUS   SYSTEM 

Diagnosis. — The  principal  diseases  from  which 
amyotcnia  has  to  be  differentiated  are  rickets  (with 
which  it  may,  of  course,  be  associated),  anterior  polio- 
myelitis, and  possibly  the  myopathies. 

From  anterior  ^poliomyelitis  it  is  distinguished  by 
its  symmetrical  distribution  and  by  the  absence  of 
complete  paralysis,  for    in    amyotonia  the  movements, 


Fig.  43.— Exti-eine  flexion  of  the  ankle-joint  in  ainyotonia  congenita. 
{From  a  case  under  the  care  of  Br.  Essex  Wynter.) 


while  they  arc  weakened,  are  not  lost.  Further,  there 
is  not  the  wasting  which  occurs  in  acute  anterior  polio- 
myelitis. 

With  the  myopathies  this  condition  has  much  in 
common,  and,  as  already  mentioned,  some  cases  are 
regarded  as  a  variety  of  that  group,  while  in  others 
lack  of  familial  tendency,  gradual  improvement,  and  the 
absence  of  pronounced  wasting  are  relied  upon  as  being 


AMYOTONIA   CONGENITA 


175 


characteristic  features  of  distinction  between  the  two 
diseases. 

Prognosis. — The  prognosis,  as  judged  from  the 
cases  hitherto  recorded,  appears  to  be  favourable.  The 
muscles   improve    as   the   child  grows,   and   eventuallv 


Fig.  it.— Grotesque  position  of  the  leg  in  tlie  same  case  as  Fig.  43. 


they  become  stronger.     In  some  instances  contractures 
have  been  observed. 

Treatment. — The  main  points  in  treating  these 
cases  are  :  (1)  to  look  after  the  general  nutrition  ;  (2)  to 
regulate  the  child's  movements  so  that  deformities  and 
ill-shapen  limbs  are  not  allowed  to  develop  from  attempt- 
ing to  walk  too  soon  ;  (3)  to  massage  and  move  the 
limbs  in  order  to  prevent  the  formation  of  contractures 
and  fixed  positions. ' 


176    DISEASES   OF  THE   NERVOUS  SYSTEM 

THE   PERONEAL   TYPE    OF   MUSCULAR 
ATROPHY 

The  early  descriptions  of  this  variety,  which  is 
characterized  by  wasting  of  the  muscles  of  the  legs  and 
forearms,  are  especially  associated  with  the  names  of 
Charcot,  Marie,  and  Tooth. 

Etiology. — Nothing  definite  is  known  of  the  causa- 
tion. The  symptoms  generally  first  appear  in  children 
or  young  adults.     There  is  a  tendency  for  several  mem- 


Fi^.  45.— Section  of  spinal  cord  showing  degenera- 
tion of  the  posterior  columns,  from  a  case  of 
peroneal  atrophy.    (Pierre  Marie.) 

bers  of  a  family  to  be  attacked,  and  cases  of  heredity 
have  occasionally  been  recorded. 

Patholofjy. — The  exact  pathology  is  still  doubtful, 
and  in  the  light  of  present  knowledge  it  is  convenient  to 
look  upon  this  variety  as  an  intermediary  form  between 
the  atrophies  of  muscular  and  those  of  spinal  origin. 
Sclerosis  of  the  posterior  (Fig.  45)  and,  to  some  extent, 
of  the  lateral  columns  of  the  cord  has  been  observed, 
and  the  occurrence  of  a  curious  hypertrophic  form  of 
neuritis  has  been  occasionally  noticed. 

Symptoms. — The  onset  is  very  gradual.  At  first 
the  peronei  and  extensors  of  the  toes  become  weak,  and 
later  on  the  calf  muscles  waste,  so  that  the  size  of  the 


PERONEAL   ATROPHY 


177 


Fig.  46.— Case  of  peroneal  atrophy.  Note 
the  wasting  of  the  muscles  below  the 
knee. 


n 


178    DISEASES   OF  THE   NERVOUS   SYSTEM 

leg  contrasts  markedly  with  that  of  the  thigh,  which 
generally  remains  normal  for  some  time  (Fig.  46). 
Clubbing  of  the  feet  occurs,  and  is  often  an  early  sign. 

When  the  thigh  muscles  remain  normal  the  knee- 
jerks  may  be  retained.  After  a  time,  wasting  takes 
place  in  the  small  muscles  of  the  hand  and  spreads  to 
the  muscles  of  the  forearm.  Deformities  resulting  in 
the  "  claw  hand  "  may  eventually  arise.  In  some  cases 
the  disease  does  not  make  any  further  progress,  but  in 
others  the  wasting  gradually  spreads  to  the  thigh  and 
trunk. 

Disturbances  of  sensation  are  uncommon,  but  pain 
and  anaesthesia  of  the  limbs  have  occasionally  been 
observed. 

Diacfuosis. — The  main  points  upon  which  reliance 
has  to  be  placed  arc  slow  symmetrical  wasting  of  the 
legs,  accompanied  by  clubbing  of  the  feet,  followed 
later  by  wasting  and  deformities  in  the  hands  and  fore- 
arms, occurring  in  children  and  young  adults,  though 
from  what  has  already  been  said  it  will  be  seen  that  cases 
may  occur  in  which  there  is  doubt  about  the  underlying 
spinal  and  neural  changes. 

Prognosis. — The  prognosis  as  regards  recovery 
is  bad,  but  as  regards  life  it  is  more  favourable  as  com- 
pared with  tlie  other  forms  of  myopathy,  for  in  the 
peroneal  type  the  atrophy  often  ceases  to  extend. 

Treatment. — Massage,  electricity,  tonics,  and  atten- 
tion to  the  general  health  are  the  only  methods  of  treat- 
ment applicable  to  this  disease. 


SECTION  IV.— DISEASES   OF  THE 
SPINAL  CORD 

CHAPTER  XVII 

CLASSIFICATION 

The  spinal  cord  begins  at  the  lower  end  of  the  medulla 
and  terminates  at  about  the  upper  border  of  the 
2nd  lumbar  vertebra,  below  which  are  the  nerve  roots 
of  the  Cauda  equina. 

In  practice  the  patient  comes  with  certain  complaints 
which  have  to  be  investigated  and  referred  to  their 
proper  causes.  But  before  this  can  be  done  it  is  neces- 
sary that  some  knowledge  should  be  acquired  of  the 
principal  lesions  which  are  likely  to  be  met  with,  and 
for  the  purpose  of  gaining  this  knowledge  classifications 
arc  utilized. 

It  is  true  that  such  classifications  are  necessarily  often 
artificial  and  not  altogether  scientific,  but  they  never- 
theless form  a  useful  working  basis  and  serve  as  an  aid 
to  memory,  w^hile  they  can  do  but  little  harm  so  long 
as  their  provisional  nature  is  duly  emphasized. 

One  such  method  of  dealing  with  diseases  of  the 
spinal  cord  is  to  classify  them  according  to  the  anatomi- 
cal distribution  of  their  lesions,  and,  taken  in  this  way, 
the  disorders  at  once  separate  themselves  into  two  large 
groups,  viz.  : 

1.  Those  in  which  the  morbid  changes  are 
confined  within  the  limits  of  certain  columns, 
i.e.  the  so-called  system  lesions  ;   and 

2.  Those  in  which  the  morbid  changes  are 
distributed  irregularly  through  the  substance  of 
the  cord  quite  irrespectively  of  column  formation, 
i.e.  non~systcin  lesions. 

179 


180    DISEASES   OF   THE   NERVOUS  SYSTEM 

System  Lesions 

System  lesions  are  produced  by  neuronic  degenera 
tion.  The  separate  columns  of  the  cord  (Plate  9)  are 
composed  of  bundles  of  neurones,  and  stress  has  already 
been  laid  on  the  fact  that  every  individual  neurone  of 
the  bundle  is  a  unit  in  itself,  and  dependent  on  its 
own  cell  body  for  its  nutrition. 

When  this  nutrition  is  impaired,  the  neurone  dies 
and  its  branches  wither  away  just  as  do  the  branches 
of  a  tree  when  something  interferes  with  its  roots. 

Certain  poisons,  of  which  that  of  syphilis  is  one  of 
the  most  important,  cause  neuronic  degeneration,  and 
the  symptoms  in  any  given  case  depend  upon  the  par- 
ticular system  or  systems  of  neurones  affected. 

Reference  to  the  accompanying  diagrams  shows  the 
chief  systems,  and  these  may  be  attacked  singly  or  in 
numbers,  and  accordingly  there  may  be  single  or  com- 
bined system  lesions. 

Of  the  single-system  lesions,  where  only  one 
column  is  attacked,  we  may  recognize  the  following  : 

1.  Disease  of  the  cells  of  the  anterior  horns, 
as  exemplified  in  acute  poliomyelitis  and  progres- 
sive muscular  atrophy  (Fig.  47). 

2.  Disease  of  the  lateral  (crossed  pyramidal) 
tracts,  as  exemplified  in  lateral  sclerosis  (Fig.  48). 

3.  Disease  of  the  posterior  columns,  as  exemplified 
ill  locomotor  ataxy  (Fig.  49). 

Mulliple-system  lesions  may  occur  as  follows  : 

1.  Disease  of  the  lateral  columns  together  with 
that  of  the  cells  of  the  anterior  horns,  as  exemplified 
in  amyotrophic  lateral  sclerosis  (Fig.  50). 

2.  Disease  of  the  lateral  columns  together  with 
that  of  the  posterior  columns,  as  exemplified  in 
ataxic  paraplegia  (Fig.  51). 

3.  Disease  of  the  lateral  columns  together  with 
that  of  the  posterior  columns,  cerebellar  tracts,  and 
(sometimes)  antero -lateral  tracts,  as  exemplified  in 
Friedreich's  disease  and  subacute  combined  de- 
generation (Fig.  52). 


GolVg 
column 


Burdach's 
column 


Marg.   zone 


Cerebellar  tract 
(Flechsig) 


Lat.  {crossed) 
pyramidal  tract 
(Flechsig) 


Gowers'  tract 


Direct  pyr.  tract  or 
Ant.  pyram. 
(Tiirck) 


Ground  bundle 

of  anterior 

column 


PLATE  9.— Tracts  of  the  spinal  cord.      {Morat.) 


SYSTEM   LESIONS 


181 


Tims  the  groundwork  of  the  combined  lesions  making 
up  the  clinical  signs  of  amyotrophic  lateral  sclerosis  and 
ataxic  paraplegia  can  be  deduced,  without  any  special 


Fig.  47. 


Fig.  49. 


Fig.  48. 


Fig.  50. 


Fig.  51. 


Fig.  52. 


effort  of  memory,  by  combining  the  symptoms  of  lateral 
sclerosis  of  the  lower  limbs  with  those  of  progressive 
muscular  atrophy  of  the  upper  limbs  in  the  case  of  the 
first,  and  with  those  of  ataxia  in  the  case  of  the  second. 


182    DISEASES   OF  THE  NEKVOUS   SYSTEM 

Irregular  or  Non-System  Lesions 
In  these   no   special  predilection  is  shown  for  the 
various  columns,  and  the  classification  has  to  be  based 
on  the  pathological  possibilities.     The  main  lesions  are  : 
Inflammations,  e.g.  myelitis — transverse  or  dis- 
seminated. 
Vascular  lesions,  e.g.  thrombosis  and  haemorrhage. 
Diffuse  and  disseminated  degenerative  processes, 

e.g.  syringomyelia  and  disseminated  sclerosis. 
Syphilitic  lesions. 
Tumours. 

Pressure  from  without,  as  in  spinal  caries, 
meningeal  disease,  injuries,  etc. 
This  method  of  classification  is,  of  course,  very  far 
from  perfect.  For  instance,  the  degeneration  in  tabes 
dorsalis  is  not  entirely  confined  to  the  posterior  columns. 
Nevertheless  the  classification  is  one  that  proves  useful 
to  students,  since  it  serves  as  a  model  which  they  can 
supplement  or  vary  at  their  discretion,  and  at  the  same 
time  it  helps  to  a  considerable  extent  to  demonstrate  the 
relationship  which  exists  between  the  different  diseases. 


CHAPTER   XVIIl 
PARAPLEGIA   AND   BULBAR    PARALYSES 

PARAPLEGIA 

Paraplegia  is  the  clinical  term  usually  applied  to  a 
condition  of  paralysis  of  both  legs,  though  it  is  equally 
applicable  when  the  arms  are  also  involved,  as,  for  in- 
stance, in  a  case  of  fracture  of  the  spine  in  the  cervical 
region. 

The  presence  of  paraplegia  indicates  that  the  trans- 
mission of  impulses  along  the  motor  fibres  has  been 
interrupted,  and  its  causes  are  best  studied  by  making 
a  diagram  of  the  motor  paths  and  then  considering  the 
whereabouts  and  nature  of  the  possible  interruptions. 

1.  Lesions  of  the  peripheral  nerves. — Peripheral 
neuritis  from  alcohol,  arsenic,  or  any  other  cause  may 
give  rise  to  complete  loss  of  power  in  both  legs,  and 
the  position  of  the  lesion  has  to  be  decided  by  the 
characteristics  of  lower  neurone  paralysis,  and  then 
differentiated  from  diseases  of  the  anterior  cornua. 

2.  Lesions  of  the  spinal  cord. — Disease  of  the 
cells  of  the  anterior  horns  in  the  lumbar  region  will,  if 
symmetrical,  cause  paraplegia. 

Here  the  paralytic  symptoms,  being  of  the  lower 
motor  neurone  type,  are  similar  to  those  of  peripheral 
neuritis,  but  there  will  be  no  sensory  changes  such  as 
occur  when  the  peripheral  nerves  are  affected.  As  a 
matter  of  fact,  a  paraplegia  of  symmetrical  distribution 
due  to  a  lesion  limited  to  the  cells  of  the  anterior  horns 
is  rare.     In  poliomyelitis  a  symmetrical  lesion  is  unusual, 

183 


i84    DISEASES   OF   THE  NERVOUS  SYSTEM 

while  in  progressive  muscular  atrophy  degeneration  of 
the  motor  cells  of  the  lumbar  region  is  seldom  a  pre- 
dominant feature. 

Any  lesion  of  the  spinal  cord  which  implicates  the 
lateral  columns  (crossed  pyramidal  tracts)  will  cause  some 
degree  of  paraplegia.      These  may  be  divided  into  : 

(a)  Those   which   occur  primarily  in  the   sub- 

stance of  the  cord. 

(b)  Those  which  press  on  the  cord  from  without. 

(a)  Those  of  the  substance  of  the  cord  may  be  trans- 
verse lesions  (complete  or  incomplete),  such  as  arise 
from  myelitis,  haemorrhages,  thrombosis,  tumours,  and 
syringomyelia,  or  they  may  be  system  lesions  in  which 
definite  columns  are  attacked,  as  in  primary  lateral 
sclerosis,  ataxic  paraplegia,  and  subacute  combined 
degeneration. 

(b)  Pressure  from  without  may  be  due  to  fracture- 
dislocation  of  the  spine,  caries,  or  tumours. 

In  all  these  cord  lesions,  except  when  the  lumbar 
enlargement  is  diseased,  the  paralysis  of  the  legs  is  that 
of  the  upper  neurone  type,  as  shown  by  the  increased 
tendon  reflexes,  rigidity,  ankle-clonus,  and  extensor 
response  of  the  plantar  reflex,  while  there  are  no  wasting 
(except  from  disuse)  and  no  changes  in  the  electrical 
reactions  of  the  muscles.  The  action  of  the  bladder 
varies  ;  when  the  lesion  extends  across  the  cord  there 
is  usually  trouble,  but  in  some  of  the  system  lesions  little 
or  nothing  may  be  noticed. 

3.  Lesions  of  the  brain,  (a)  Subcortical. — ^As  we 
pass  above  the  cord  the  motor  fibres  diverge  to  the 
cortex  of  the  brain,  and  therefore  between  the  region 
of  the  pons  and  the  cortex  it  is  unlikely  that  any  single 
lesion  would  injure  them  on  both  sides.     (Plate  1.) 

Symmetrical  lesions  are  naturally  uncommon,  hence 
hemiplegia  rather  than  paraplegia  is  the  usual  result 
of  disease  in  this  area  ;  but  it  does  occasionally  happen 
that  two  vascular  lesions  succeed  one  another  and  cause 
a  "  double  hemiplegia,"  or  "  diplegia,"  which  is  to  all 
intents  and  purposes  a  paraplegia. 


PARAPLEGIA  185 

(6)  Cortical. — in  the  cortex  the  motor  areas  for  the 
legs  are  not  far  from  each  other,  and  any  injury  to  the 
vertex  of  the  brain  is  liable  to  damage  both  the  leg 
centres  and  cause  paraplegia.  Some  of  the  paraplegias 
(known  as  diplegias)  of  infancy  arise  in  this  way  through 
injuries  at  birth  {see  Cerebral  Diplegia,  p.  330),  but  it  is 
seldom  that  paraplegia  arises  in  this  manner  in  later  life. 

(c)  Hysteria. — Paraplegia  of  a  most  complete  type 
may  be  of  hysterical  origin,  and  its  cause  is  then  prob- 
ably situated  in  the  cerebral  cortex. 

Some  of  these  hysterical  cases  give  rise  to  great 
difficulty  in  diagnosis,  for  though  they  can  usually  be 
easily  distinguished  from  lower  neurone  lesions,  such  as 
neuritis,  where  the  wasting  and  the  loss  of  reflexes  make 
the  condition  clear,  they  are  often  confused  with  cord 
lesions,  and  especially  with  those  due  to  commencing 
disseminated  sclerosis,  in  which  the  symptoms  are  apt 
to  vary  in  the  same  extraordinary  fashion  as  they  do  in 
functional  disease. 

It  is  in  these  doubtful  cases  that  the  extensor  response 
of  the  plantar  reflex  is  so  valuable,  for  increased  knee- 
jerks,  with  perhaps  slight  ankle-clonus,  may  be  present 
in  both. 

Further  particulars  of  differential  diagnosis  of  dis- 
seminated sclerosis  will  be  found  at  p.  216. 

For  the  prognosis  of  paraplegia  reference  may  be 
made  to  the  chapters  dealing  with  the  different  diseases 
in  which   paraplegia  occurs. 

Senile  Paraplegia 
In  elderly  people  some  weakness  of  the  legs,  accom- 
panied, perhaps,  by  difficulty  in  exercising  full  control 
over  the   sphincters,   may  occur  from  anaemia  of  the 
cord  due  to  atheroma  of  the  spinal  arteries. 

Intermittent  Claudication 
Another  difficulty  in  walking — also  due,  it  is  sup- 
posed, to  atheroma — ^is  that  known  under  the  name  of 
intermittent  claudication,  or  lim'ping^  and  characterized 


186    DISEASES   OF  THE  NEKVOUS  SYSTEM 

by  sudden  attacks  of  lameness,  often  accompanied  by 
cramps,  blueness  of  the  extremities,  and  sensations  of 
tingling.  After  walking  a  variable  distance  the  patients 
are  pulled  up  suddenly  with  cramp,  which  usually  passes 
off  with  rest.  The  symptoms  in  these  cases  are  fre- 
quently attributable  to  impaired  nutrition  of  the  muscles 
consequent  on  the  arterial  disease,  and  absence  of 
arterial  pulsation  and  the  presence  of  gangrene  in  the 
feet  may  be  associated  conditions.  In  other  instances 
the  trouble  may  be  secondary  to  lesions  of  the  cord  or 
brain. 

BULBAR    PARALYSES 

Paralyses  of  bulbar  origin,  in  which  the  tongue, 
pharynx,  and  larynx  are  affected,  may,  like  other  para- 
lyses, be  of  upper  or  lower  neurone  type.     (Fig.  53.) 

1.  Upper  neui'one  type. — In  this  form  the  motor 
fibres  are  interrupted  in  their  course  from  the  cortex 
to  the  medulla,  but,  in  order  to  produce  any  permanent 
effect  on  the  bulbar  functions,  the  interruption  must 
be  bilateral.  A  unilateral  lesion,  e.g.  a  haemorrhage  into 
the  internal  capsule,  though  it  may  affect  the  movements 
of  one  half  of  the  tongue  and  palate,  will  not  as  a  rule 
cause  any  permanent  loss  of  articulation  and  difficulty 
in  swallowing.  On  the  other  hand,  a  bilateral  lesion, 
by  cutting  off  the  motor  fibres  from  both  sides  of  the 
cortex,  will  deprive  the  nuclei  of  voluntary  innervation, 
and  in  such  a  case  the  movements  of  the  tongue,  palate, 
and  larynx  may  be  greatly  impaired. 

Bilateral  lesions  of  cortical  origin  may  be  seen  in 
severe  cases  of  cerebral  diplegia  in  children  ;  they  also 
occur  in  amyotrophic  lateral  sclerosis  and  in  cases  where 
a  haemorrhage  in  the  region  of  the  internal  capsule  is 
followed  by  another  in  a  similar  position  on  the  opposite 
side.  The  bulbar  symptoms  due  to  upper  neurone  lesions 
are  often  termed  fseudo-bulbar  'paralysis,  to  distinguish 
them  from  the  lower  neurone  lesions  in  which  the  func- 
tions of  the  nuclei  themselves  are  lost. 

The  same  rules  for  upper  motor  neurone  lesions  apply 
here  as  elsewhere.     The  power  of  voluntary  movement 


BULBAR   PARALYSES 


187 


is  diminished.  There  is  inability  to  pout  the  lips  in 
order  to  blow  or  whistle,  the  tongue  cannot  be  protruded 
or  thrust  into  the  cheeks,  and  articulation  is  very  in- 
distinct. Mastication  and  swallowing  become  difficult, 
the  saliva  dribbles  out  between  the  paralysed  lips,  and, 
in  swallowing,  fluid  may  regurgitate  through  the  nose. 
The  reflexes  of  the  palate,   pharynx,   and  larynx  are 


i-'BILATERAL     CEREBRAL 
LESIONS 


"LESIONS   AT   THE 
NUCLEI 


Fig. 


■-'-LESIONS     OF  THE 
NERVES    ARISING 
FROM  THE  NUCLEI 


53.— Diagram  showing  different   situations   in  which 
bulbar  paralyses  may  be  produced. 


preserved,  a  jaw-clonus  may  be  present,  and  there  are 
no  wasting  and  no  changes  in  the  electrical  reactions 
of  the  paralysed  muscles. 

2.  Lower  neurone  lesions. — Here  the  disease  may 
be  in  the  nuclei  or  in  the  nerves  arising  from  them.  The 
nuclei  of  the  bulb,  from  which  the  10th,  11th,  and  12th 
nerves  arise,  are  subject  to  the  same  diseases  as  nuclei 
elsewhere.     They  may  be  grouped  as  follows  : 


188    DISEASES   OF  THE   NERVOUS   SYSTEM 

(1)  Inflammations. — Acute  inflammatory  affections  of 
the  bulbar  nuclei  are  imcommon,  but  acute  polio- 
encephalitis, which  is  a  condition  analogous  to  that 
of  anterior  poliomyelitis  (infantile  paralysis)  in 
the  cord,  and  lethargic  encej^halitis  may  both 
occur  in  this  region. 

(2)  Degenerations.  —  Chronic  degeneration  of  the 
bulbar  nuclei  constitutes  the  disease  known  as 
progressive  bulbar  paralysis.  This  condition  is  in 
reality  a  part  of  progressive  muscular  atrophy, 
and  is  described  under  the  heading  of  that  disease 
(Chapter  XXIV.,  p.  219). 

(3)  Vascular  lesions. — {a)  Haemorrhage,  (6)  throm- 
bosis, (c)  embolism.  Haemorrhage  of  any  size 
into  the  bulb  naturally  soon  causes  death,  but 
thrombosis  or  embolism,  more  especially  throm- 
bosis of  some  small  branches,  may  cause  symptoms 
of  disease  of  the  bulbar  nuclei.  The  more  sudden 
onset  of  the  symptoms  serves  to  distinguish  such 
cases  from  the  progressive  bulbar  variety  ;  their 
course  depends  upon  the  temporary  or  permanent 
effects  which  the  lesion  produces. 

(4)  Pressure. — Tumours  in  the  neighbourhood  of 
the  medulla  may  give  rise  to  bulbar  symptoms 
from  pressure. 

When  the  bulbar  paralysis  is  due  to  injury  of  the 
nerves  after  they  have  left  the  nuclei  the  symptoms  are 
generally  unilateral  ;  moreover,  the  spinal  portion  of  the 
spinal  accessory  will  in  all  probability  be  also  injured,  in 
consequence  of  which  the  additional  symptoms  of  paraly- 
sis of  the  sterno-mastoid  and  trapezius  will  be  present. 

Examples  of  neuritis  in  which  the  symptoms  are  sym- 
metrical and  of  bulbar  distribution  are  met  with  in  cases 
of  postdiphtheritic  paralysis.  Another  disease  in  which 
bulbar  signs  are  also  often  prominent  is  myasthenia 
gravis,  the  pathology  of  which  is  at  present  not  certainly 
known,  though  there  is  some  evidence  to  show  that  the 
morbid  changes  may  possibly  commence  in  the  muscles 
themselves  {see  Chapter  XLIIL,  p.  421). 


CHAPTER   XIX 

ACUTE   POLIOMYELITIS    (INFANTILE    PARALYSIS) 

Acute  poliomyelitis  (known  clinically  also  as  infantile 
paralysis)  is  the  result  of  acute  inflammatory  changes 
taking  place  in  the  motor  cells  of  the  anterior  horns  of 
the  spinal  cord. 

Etiology. — ^Acute  poliomyelitis  is  a  contagious  dis- 
ease which  may  spread  from  person  to  person  and  may 
occur  sporadically  or  in  epidemic  form. 

The  precise  method  by  which  the  contagion  is  con- 
veyed is  still  uncertain,  but  there  is  evidence  to  show 
that  it  can  be  carried  by  healthy  persons,  so  that  the 
infected  patient  need  not  necessarily  have  been  in 
direct  contact  with  anyone  suffering  from  the  disease. 
Most  of  the  epidemics  occur  in  the  summer  and  autumn, 
showing  that  the  virulence  of  the  infection  is  subject 
to  seasonal  influence.  Flexner  and  Lewis,*  following 
the  earlier  experiments  of  Landsteiner  and  Popper, 
succeeded  in  transmitting  the  disease  to  monkeys, 
and  as  the  result  of  these  and  other  experiments 
it  has-been  shown  (1)  that  the  virus  has  a  special  pre- 
dilection for  the  spinal  cord  ;  (2)  that  its  infective 
power  shows  a  considerable  resistance  to  glycerin  and 
to  degrees  of  temperature  that  usually  affect  poisons  of 
a  similar  nature ;  and  (3)  that  it  is  one  of  those  organisms 
which  pass  through  bacterial  filters. 

Hitherto  all  attempts  to  obtain  a  culture  of  the 
specific  organism  have  failed,  though  more  recently 
NogLichi  is  said  to  have  isolated  it. 

*  ' '  Epidemic  Poliomyelitis. ' '     New  York,  1910. 
189 


190    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  length  of  the  incubation  period  is  uncertain, 
but  it  is  thought  to  vary  from  three  to  ten  days.  The  dis- 
ease is  especially  liable  to  attack  the  young,  and  although 
adults  are  by  no  means  immune  the  statistics  of  epidemics 
show  that  it  is  mainly  a  disease  of  childhood. 

As  already  mentioned,  the  virus  tends  to  affect  the 
spinal  cord,  and  it  specializes  this  tendency  by  the 
manner  in  which  it  attacks  the  motor  cells  of  the  anterior 
horns.  Careful  study  of  the  symptomatology  as  it 
occurs  in  large  epidemics  has,  however,  shown  that 
the  effects  of  the  poison  are  by  no  means  always  con- 
fined to  this  part  of  the  cord,  and  so  it  has  been 
necessary  to  broaden  the  clinical  conceplion  of  this 
disease  and  to  recognize  that  the  virus  w^hich  is 
common  to  many  cases  does  not  produce  in  all  of 
them  the  symptoms  that  were  formerly  thought  to  be 
characteristic. 

Since  in  these  epidemics  the  symptoms  of  different 
cases  are  apt  to  differ  in  such  a  manner  as  to  make  it 
difficult  to  form  them  into  a  common  group  of  what  was 
clinically  recognized  as  acute  poliomyelitis,  it  has  been 
proposed  to  term  the  epidemic  form  Heine-Medin's 
disease,  after  Heine  who  gave  one  of  the  earliest  descrip- 
tions of  the  disease,  and  Medin  who  made  important 
observations  on  its  epidemic  form. 

Patholocjy. — In  an  acute  case,  redness  and  swelling 
of  the  grey  matter  of  the  spinal  cord  can  often  be 
seen  with  the  naked  eye.  Microscopically,  acute  hypcr- 
aemia,  inflammatory  exudation,  and  disintegration  of  the 
motor  cells  of  the  anterior  horns  are  among  the  principal 
changes  observed.  The  cell  bodies  are  swollen,  their 
Nissl  granules  broken  up,  and  their  nuclei  displaced  or 
disorganized. 

Capillary  haemorrhages,  thrombosis  of  vessels,  and 
oedema  of  the  cord  may  also  be  observed. 

The  changes  are  generally  most  intense  in  the  grey 
matter  of  the  anterior  horns,  but  they  may  often  be  seen 
to  extend  into  the  posterior  horns  and  occasionally  even 
into  the  white  matter. 


ACUTE    POLIOMYELITIS  191 

There  is  also  infiltration  of  the  pia  mater,  and  this 
probably  explains  the  presence  of  the  meningeal  symp- 
toms that  are  found  in  many  cases. 

The  route  by  which  the  virus  enters  the  body  is 
not  known,  but  once  in  the  system  there  is,  from  experi- 
mental evidence,  good  reason  to  believe  that  it  reaches 
the  cord  through  the  lymphatics  and  not  through  the 
blood,  and  while  the  special  vascular  supply  of  the 
anterior  horns  may  have  some  influence  in  determining 
the  localization  of  the  disease,  it  is  evidently  not  the 
deciding  factor,  as  it  was  formerly  thought  to  be.  It 
is  generally  conceded  that  thrombosis  and  embolism  are 
secondary  effects.  The  cerebro-spinal  fluid  is  some- 
times clear  and  at  other  times  shows  lymphocytosis. 

Enlargement  of  the  spleen  and  swelling  of  the 
lymphoid  tissue  of  the  alimentary  canal  may  occur, 
and  are  probably  the  results  of  a  generalized  infection, 
though  by  some  the  alimentary  changes  are  considered 
as  a  possible  indication  of  the  path  of  entry  of  the  virus. 

In  the  later  stages,  after  the  vascular  disturbance 
has  subsided,  the  chief  features  are  disappearance  and 
disintegration  of  motor  cells,  with  an  excess  of  connective 
tissue  that  has  taken  their  place. 

Symptoms. — The  early  symptoms  are  those  of  a 
general  toxaemia.  The  child  becomes  fretfnl  and  rest- 
less, and  in  some  cases  drowsiness  is  a  prominent  feature. 
The  temperature  is  raised  and  convulsions  have  been 
known  to  occur,  but  they  are  unusual  unless  encephalitis 
is  also  present.  The  child  dislikes  being  touched  or 
moved,  and  seems  to  be  in  pain.  Sweating  and  diarrhoea 
are  often  present.  In  the  course  of  a  day  or  two  it  is 
noticed  that  one  or  more  of  the  limbs  are  not  being 
freely  used.  This  generally  gives  rise  to  alarm  and  leads 
to  the  examination  of  the  child,  when  the  most  char- 
acteristic feature  of  this  stage  of  the  disease — 'presence 
of  a  flaccid  paralysis  of  acute  onset  and  irregular  distri- 
bution— 'is  revealed. 

Groups  of  muscles  are  picked  out  irregularly  in 
different  limbs,  but  the  lower  limbs  are  affected  more 


192     DISEASES   OF  THE  NERVOUS   SYSTEM 

frequently  than  the  upper,  and  the  quadriceps  extensor, 
tibialis  anticus,  and  peronei  seem  especially  prone  to  be 
attacked.  The  reflexes  in  the  affected  areas  are  generally 
lost.  The  muscles  of  the  trunk,  including  the  intercostals, 
may  be  paralysed  (Fig.  54),  and  the  respiratory  move- 
ments may  be  still  more  seriously  impaired  through 
paresis  of  the  diaphragm.  It  is  not  uncommon  to  find 
that  muscles  supplied  by  the  cranial  nerves  are  affected  ; 
and  a  bulbar  type  in  which  the  nuclei  of  the  medulla  are 
affected  may  also  occur. 

It  soon  becomes  apparent  that  the  paralysed  muscles 
are  rapidly  wasting,  and  at  the  end  of  ten  days  or  a  fort- 
night their  electrical  reactions,  if  tested,  will  probably 
be  those  of  degeneration. 

In  a  typical  case  there  is  no  disturbance  of  sensation 
and  usually  no  loss  of  control  over  the  sphincters. 

In  those  cases  where  the  effects  of  the  \arus  are  not 
confined  to  the  cells  of  the  anterior  horn  the  symptoms 
do  not  conform  accurately  to  the  average  type.  This  is 
the  explanation  of  cases  in  which  some  alteration  in 
sensation  has  been  observed,  and  of  others  in  which 
the  pyramidal  columns  have  been  affected  in  addition 
to  the  anterior -horn  cells,  but  such  instances  are  un- 
common and  must  be  regarded  as  exceptional. 

Clinical  varieties. — Many  clinical  varieties  are 
met  with,  according  to  the  parts  of  the  cord  that  are 
attacked. 

One  of  the  most  important  is  the  form  which  resembles 
Landry's  paralysis,  with  which  indeed,  in  some  cases 
at  least,  it  may  be  identical.  Here  the  paralysis  spreads 
from  the  feet  upwards  with  no  sensory  disturbance  and 
with  no  appreciable  wasting  of  muscles,  giving  all  the 
appearance  of  greatly  impaired  function  of  the  motor 
cells  stopping  short  of  their  actual  destruction. 

There  is  also  a  form  which  closely  resembles  poly- 
neuritis, and  in  other  instances,  there  may  be  symptoms 
of  ataxy. 

The  virus  may  also  attack  the  brain  and  produce 
encephalitis,  a  description  of  which  is  given  at  p.  341. 


ACUTE    POLIOMYELITIS 


193 


Diagnosis. — The  acute  onset  with  rise  of  tempera- 
ture and  wasting  of  muscles  is  very  characteristic  of  the 
disease.  When  there  is  much  pain  the  symptoms  may  at 
first  resemble  those  of  spinal  meningitis,  but  as  the  disease 
progresses  and  wasting  ensues  the  difierence  usually  soon 
becomes  apparent.     As  the  febrile  symptoms  pass  off. 


Fig.  54.— "Wasting  and  deformities  following  widespread 
acute  poliomyelitis. 


loss  of  power  becomes  the  prominent  feature  and  the 
wasting  of  the  muscles  and  grouping  of  the  paralysis,  to- 
gether with  changes  in  the  electrical  reactions,  show  that 
it  is  the  lower  motor  neurone  which  is  at  fault,  i.e.  that 
the  lesion  is  situated  somewhere  between  the  cells  of  the 
anterior  horns  and  the  muscles.  As  acute  diseases  of  the 
muscles,  giving  rise  to  such  symptoms,  are  unknown,  the 
issue  is  in  most  cases  further  narrowed  between  diseases 


194    DISEASES   OF   THE   NERVOUS   SYSTEM 

of  tlie  peripheral  nerves  and  of  the  anterior  horns. 
Peripheral  neuritis  can  generally  be  excluded  by  the 
absence  of  pain  and  of  sensory  disturbance,  and  by  the 
irregular  distribution  of  the  paralysis  and  its  failure  to 
correspond  to  the  muscle  areas  of  peripheral  nerves. 
Moreover,  most  cases  of  multiple  neuritis  of  toxic  origin, 
e.g.  those  due  to  alcohol,  arsenic,  and  lead,  develop  so 
deliberately  that  there  is  little  chance  of  their  being  mis- 
taken for  acute  poliomyelitis,  while  that  due  to  diphtheria, 
where  the  onset  might  be  more  confusing,  usually  has 
the  typical  distribution  of  paralysis  of  the  soft  palate  and 
ocular  muscles.  Difhculty,  however,  occasionally  arises 
in  cases  of  multiple  neuritis  of  acute  onset  in  which  no 
definite  changes  in  sensation  can,  at  any  rate  for  a 
time,  be  demonstrated,  but  here  again  the  symmetry 
is  of  great  assistance. 

Lesions  of  the  muscles  and  peripheral  nerves  being 
excluded,  the  cells  of  the  anterior  horns  remain  to  be 
considered.  Here  the  only  conditions  that  are  likely 
to  occasion  confusion  are  those  in  which  the  function  of 
the  cells  is  very  rapidly  modified  by  toxins  that  give 
rise  to  cases  included  in  the  clinical  group  of  Landry^s 
paralysis.  Here  the  patient  rapidly  loses  power,  as  in 
acute  poliomyelitis,  but  there  is  usually  no  definite  wast- 
ing of  muscles  nor  are  there  changes  in  their  electrical 
reactions.  Moreover,  the  loss  of  power  in  Landry's 
paralysis  advances  evenly  upwards  from  the  feet,  while 
that  of  acute  poliomyelitis  is  nearly  always  irregularly 
distributed. 

While  in  clinical  appearance  and  course  this  variety 
differs  from  the  typical  "  infantile  paralysis,"  it  is 
still  probable,  as  already  stated,  that  the  under- 
lying virus  of  both  Landry's  paralysis  and  polio- 
myelitis is,  at  any  rate  sometimes,  the  same,  since 
during  epidemics  the  two  types  may  be  found  side 
by  side. 

Prognosis. — The  prognosis  has  to  be  considered 
(1)  as  regards  life  and  (2)  as  regards  recovery  of 
power. 


ACUTE    POLIOMYELITIS  195 

1.  Danger  to  life. — From  a  study  of  epidemics  it  is 
now  recognized  that  the  danger  to  life  is  considerable. 
The  mortality  differs  widely  in  different  epidemics,  in 
some  being  as  low  as  2  and  in  others  as  high  as  20  per 
cent,  and  even  higher,  and  it  is  greater  in  adults  than 
in  children. 

Cases  with  bulbar  symptoms  are  always  serious,  as 
also  are  those  which  exhibit  the  symptoms  of  Landry's 
paralysis,  since  in  these  latter  the  respiratory  move- 
ments are  particularly  liable  to  be  affected. 

2.  Recovery  of  foiver. — In  some  cases  the  illness 
consists  almost  entirely  of  general  toxsemic  symptoms, 
the  local  signs  being  absent  or  so  slight  that  no  anxiety 
arises  as  to  any  permanent  paralysis.  In  most  cases, 
however,  there  is  the  loss  of  power  that  is  so  character- 
istic of  the  disease,  and  in  these  recovery  is  seldom 
anything  like  complete,  although  there  is  often  very 
considerable  improvement. 

The  guide  to  the  prognosis  in  these  cases  is  to  be 
found  in  a  consideration  of  their  morbid  anatomy. 

If  a  group  of  cells  taken  from  an  affected  area 
be  examined,  it  will  be  seen  that  some  are  entirely 
destroyed,  others  are  badly  injured,  while  still  others 
on  the  borders  of  the  diseased  area  have  escaped. 
Those  that  are  destroyed  can  never  recover  their  func- 
tion, but  those  in  which  the  injury  is  less  complete 
are  capable  of  some  degree  of  regeneration,  and  it  is 
upon  these  latter  that  the  ultimate  degree  of  recovery 
largely  depends. 

As  a  general  rule,  it  may  be  said  that  if  all  goes 
well  some  considerable  degree  of  improvement  will 
take  place — ^but  as  to  how  much,  time  alone  can  show. 
When  the  paralysis  is  extensive  the  growth  of  all  the 
structures  will  be  impaired.  The  limb  remains  stunted, 
wasted,  and  colder  than  its  fellow,  and  troublesome 
ulcers  may  form  in  the  skin.  As  time  goes  on,  the 
muscular  tissues  contract  and  produce  various  kinds  of 
talipes  in  the  leg  or  corresponding  deformities  in  the 
arm  (Fig.  55). 


196    DISEASES   OF  THE  NERVOUS   SYSTEM 

Treatment.  (1)  Prophylactic— Since  the  disease  is 
known  to  be  contagious,  it  is  desirable  to  isolate  the 
patients  and  to  disinfect  the  intestinal  excreta  and  any 
discharge  from  the  nose  or  other  mucous  channels  in 
which  the  virus  may  be  contained.     The  length  of  time 


Fig.  55.— Wasting  aud  deformity  of  the  right  hand  and  arm  as 
the  result  of  acute  poliomyelitis  in  childhood. 

required  for  isolation  to  be  successful  is  uncertain,  and  has 
been  placed  at  periods  ranging  from  three  to  eight  weeks. 
Unfortunately,  isolation  cannot  be  depended  upon 
to  check  the  spread  of  the  disease,  the  virus  of  which, 
AS  already  mentioned,  is  apparently  often  conveyed  by 
healthy    intermediaries,    and    moreover    in    epidemics 


ACUTE   POLIOMYELITIS  197 

there  are  frequently  abortive  cases  which  remain  un- 
recognized and  serve  as  centres  of  fresh  infection. 

(2)  Therapeutic. — During  the  febrile  stage  the  child 
should  be  kept  in  bed.  The  bowels  should  be  opened 
by  a  dose  of  grey  powder  or  calomel,  repeated  if  necessary, 
and  an  antifebrile  mixture  containing  diaphoretics  and 
diuretics  may  be  given.  SaHcylates,  aspirin,  and  phena- 
cetin  are  among  the  drugs  frequently  prescribed.  At  this 
stage  little  more  can  be  done,  but  as  soon  as  the  acute 
symptoms  have  subsided  efiorts  must  be  made  to  main- 
tain the  nutrition  of  the  muscles  until  the  damaged  cells 
recover  sufficiently  to  resume  their  functions.  Massage 
and  passive  movements  are  generally  considered  the  most 
reliable  methods,  together  ^\ith  electrical  treatment. 
Nowhere  is  perseverance  in  treatment  more  necessary 
than  in  this  disease.  The  treatment  must  be  kept 
up  for  a  very  long  time,  with  the  object  of  improving 
those  fibres  which,  though  badly  nourished,  are  still, 
by  persistent  stimulation,  capable  of  being  strengthened 
sufficiently  to  aid  in  moving  the  limb.  During  this 
period  every  effort  must  be  made  to  prevent  contrac- 
tures and  deformities,  by  means  of  light  massage,  passive 
movements,  and  the  judicious  use  of  splints.  Above 
all,  the  limb  must  never  be  allowed  to  assume  such 
a  position  as  to  cause  tension  or  stretching  of  the  para- 
lysed muscles,  but  must  always  be  supported  in  such 
a  manner  as  to  keep  these  muscles  slack.  Sir  Eobert 
Jones  a  long  time  ago  drew  attention  to  the  retardation 
of  recovery  which  takes  place  in  paralysed  muscles  that 
are  overstretched,  and  he  showed  that,  in  the  case  of 
infantile  paralysis,  any  statement  concerning  possibilities 
of  recovery  must,  to  be  accurate,  be  based  upon  an  esti- 
mate as  to  how  much  of  the  muscle  paralysis  is  due  to 
overstretching  and  how  much  to  complete  destruction 
of  the  corresponding  cells  in  the  spinal  cord. 

(3)  Operative. — When  the  limits  of  improvement  seem 
to  be  reached,  or  when  there  are  signs  of  deformity,  the 
orthopaedic  surgeon  may  be  consulted  with  a  view  to 
improving  the  usefulness  of  the  limb  by  operation. 


CHAPTER   XX 

LANDRY'S    PARALYSIS 

This  disease,  first  described  by  Landry,  is  characterized 
clinically  by  a  paralysis  which  begins  in  the  feet  and 
rajoidly  extends  upwards  to  the  triink,  arms,  and  bulb  ; 
occasionally  the  parts  may  be  invaded  in  the  opposite 
order.  Both  sexes  may  be  attacked,  and  most  of  the 
cases  have  occurred  between  the  ages  of  20  and  45. 

Etiology. — There  can  be  little  doubt  that  the 
symptoms  are  the  result  of  poisons  affecting  the  functions 
of  the  spinal  cord,  and  sometimes  they  have  appeared 
to  be  the  direct  sequelae  of  attacks  of  septicaemia,  typhoid, 
influenza,  and  other  specific  fevers.  There  is,  however, 
reason  for  thinking  that  in  some  cases  the  underlying 
toxin  is  similar  to,  if  not  identical  with,  that  of  acute 
poliomyelitis,  since  the  symptoms  of  both  diseases  are 
met  with  in  epidemics  of  acute  poliomyelitis  {see  p.  194). 

Pathology. — No  definite  changes  in  the  cord  can  be 
seen  with  the  naked  eye.  Microscopically,  hypersemia 
and  swelling  of  the  cells  have  been  observed.  Changes 
in  the  peripheral  nerves  have  also  been  described.  The 
loss  of  function  seems  to  be  in  considerable  advance 
of  any  definite  changes  in  structure. 

Syiriptonis. — Weakness  begins  in  the  legs,  usually 
in  one  a  few  hours  before  the  other,  and  in  a  very  short 
time  there  is  complete  flaccid  paralysis.  The  loss  of 
power  spreads  upwards  to  the  muscles  of  the  abdomen, 
thorax,  arms,  neck,  and  face. 

The  muscles  are  flaccid,  but  usually  show  no  definite 
changes  in  their  electrical  reactions  and  little  or  no  signs 

198 


LANDRY'S   PARALYSIS  199 

of  wasting.  The  tendon  reflexes  are  lost,  but  the  power 
of  the  sphincters  is  generally  retained.  As  soon  as  the 
paralysis  spreads  into  the  cervical  region  the  patient 
is  in  imminent  danger  of  death  from  paralysis  of  the 
diaphragm ;  and  if  he  escapes  this  danger,  another  is 
frequently  met  with  from  failure  of  the  muscles  of 
deglutition,  with  a  consequent  tendency  for  food  to  find 
its  way  into  the  larynx.  Sensation  is  but  little,  if  at  all, 
altered,  and  the  mind  remains  clear.  The  temperature 
may  be  raised,  but  pyrexia  is  not  a  prominent  feature. 
The  sj^leen  is  generally  enlarged. 

Variations. — Inasmuch  as  the  nature  of  the  poison 
is  probably  not  the  same  in  all  cases,  and  the  anatomical 
changes  vary,  it  is  not  surprising  that  all  cases  do  not 
adhere  rigidly  to  the  one  type.  In  some  the  paralysis 
appears  first  at  the  upper  end  of  the  body  and  spreads 
downwards  ;  in  others,  especially  those  w^hich  last  a  long 
time,  there  may  be  signs  of  wasting  of  the  muscles 
and  changes  in  their  electrical  reactions ;  while 
in  others  again  the  action  of  the  sphincters  may  be 
impaired. 

Nevertheless,  there  is  clinically  a  very  definite  set 
of  cases  characterized  by  a  paralysis  which  ascends 
and  which  is  unaccompanied  by  wasting  or  by  sensory 
disturbances. 

The  most  practical  view  is  to  suppose  that  certain 
poisons,  under  certain  conditions,  lower  the  functions 
of  the  cells  of  the  anterior  horns  (and  perhaps  other  parts 
of  the  nervous  system  as  well)  sufiiciently  to  produce  a 
flaccid  paralysis,  but  not  sufiiciently  to  cause  wasting 
and  electrical  changes  ;  that  here  and  there  the  poison 
is  intense  enough  to  lead  to  such  changes,  and  that 
gross  anatomical  lesions  sometimes  further  intensify  the 
symptoms.  In  this  way  we  can  understand  the  variation 
of  the  symptoms  in  different  cases. 

Diagnosis. — The  definite  march  of  the  paralysis, 
and  the  absence  of  wasting,  of  electrical  changes,  and  of 
altered  sensation  generally,  enable  the  disease  to  be 
distinguished,  though,  from  what  has  already  been  said, 


200    DISEASES   OF   THE  NERVOUS   SYSTEM 

it  will  be  plain  that  the  less  typical  cases  may  occasion 
difficulty; 

From  peripheral  neuritis  Landry's  paralysis  differs 
in  the  extension  of  the  paralysis  to  the  trunk  before  the 
arms  are  affected,  and  also  in  the  absence  of  wasting 
and  of  electrical  clianges,  which  are  generally  soon  seen 
in  peripheral  neuritis. 

In  an  ascending  myelitis,  where  there  is  a  definite 
inflammation  spreading  up  the  cord,  there  will  be  loss 
of  control  over  the  sphincters,  and  bedsores  wull  soon 
occur. 

An  infantile  paralysis  (acute  anterior  poliomyelitis) 
in  which  the  symptoms  are  widespread  may  be  difficult 
to  differentiate  for  a  time,  since  both  this  and  Landry's 
paralysis  depend  upon  lesions  of  the  motor  cells  of  the 
anterior  horns.  As  already  stated,  some  cases  are 
apparently  only  different  clinical  manifestations  of  the 
same  infection.  Usually  the  more  scattered  paralysis  of 
the  poliomyelitis  makes  the  diagnosis  clear,  but  where 
there  is  a  difficulty  it  may  be  necessary  to  wait  until 
wasting  and  electrical  changes  appear  in  the  muscles. 
When  a  patient  recovers  from  Landry's  disease  the 
power  returns  more  rapidly  than  in  recovery  from 
infantile  paralysis. 

Prognosis. — A  patient  suffering  from  Landry's  dis- 
ease is  always  in  great  danger  until  the  acute  symptoms 
have  subsided  and  the  risk  of  respiratory  failure  has 
passed. 

In  a  large  number  of  cases  the  disease  ends  fatally 
in  a  few  days,  while  in  some  the  symptoms  subside  and 
power  gradually  but  steadily  returns. 

Treatmeut. — In  the  absence  of  any  specific  antidote 
to  the  poison,  rcHance  has  to  be  placed  on  maintaining 
the  patient's  strength,  and  for  this  purpose  hypodermic 
injections  of  strychnine  are  probably  the  best.  Ergot 
lias  been  recommended. 

The  breathing  of  the  patient  should  be  carefully 
watched,  and  an  attempt  be  made  to  tide  over  any 
temporary  difficulty  by  artificial  respiration. 


CHAPTER   XXI 

MYELITIS 

Etiology. — The  nature  of  the  lesions  whicli  are  included 
under  the  clinical  term  "  myelitis  "  differs.  In  some 
cases  there  is  undoubtedly  a  primary  inflammation 
brought  about  by  the  direct  invasion  of  bacilli  or  their 
toxins  through  the  blood-  or  the  lymph-stream ;  in  others 
the  underlying  condition  is  one  of  thrombosis,  to  which 
the  changes  in  the  cord  are  secondary. 

It  will  therefore  be  evident  that  infective  diseases 
and  syphilitic  endarteritis  axe  the  most  common  causes. 
Cold,  injury,  and  debility  from  any  cause  may,  by 
lowering  the  resistance,  dispose  to  an  attack. 

The  most  vulnerable  part  of  the  cord  appears  to 
be  the  lower  dorsal  region,  and  it  is  there  that  the  lesion 
most  frequently  occurs. 

Pathology. — The  substance  of  the  cord  is  softened, 
and  in  some  cases  the  different  structures  are  indis- 
tinguishable from  one  another.  Haemorrhages,  engorge- 
ment of  vessels,  exudation,  and  cell  degeneration  are 
all  present  in  varying  degree.  In  the  more  chronic 
cases  the  distribution  differs  according  as  the  infection 
arrives  by  way  of  the  blood  or  of  the  lymphatics 
{see  p.  72). 

Symptoms. — The  symptoms  vary  with  the  level 
and  the  extent  of  the  lesion. 

1.  Transverse  dorsal  myelitis. — When  the  onset  is  acute, 
and  the  patient  has  hitherto  been  well,  the  first  signs 
are  generally  a  feeling  of  increasing  heaviness  in  the 
limbs,   with   perhaps   some   difficulty  in  passing  urine. 

201 


202    DISEASES   OF   THE  NERVOUS   SYSTEM 

The  numbness  and  weakness  rapidly  increase,  and  in 
a  severe  case  the  lower  limbs  may  become  within  a  short 
time  both  senseless  and  motionless.  Examination  shows 
loss  of  'power  below  the  level  of  the  lesion,  and,  assuming 
the  attack  to  be  severe,  there  will  be  absence  of  all 
movements  of  the  feet,  knees,  and  hips,  the  limbs  lying 
flaccid  and  helpless.  At  this  early  stage  the  tendon 
reflexes  may  possibly  be  temporarily  inhibited,  but 
they  Koon  become  increased,  as  in  other  upper  motor 
neurone  lesions.  At  the  same  time  that  impulses  are 
prevented  from  flowing  down  the  motor  tracts,  the 
transverse  lesion  also  prevents  stimuli  from  j^^ssing 
up  the  sensory  columns  ;  hence  in  severe  cases  there 
is  loss  of  sensation  of  every  kind  below  the  level  of  the 
lesion,  but  at  that  level,  and  just  above  it,  there  is 
often  a  zone  of  hyperesthesia,  due  to  irritation  of  the 
sensory  roots  as  they  pass  out  from  those  segments 
of  the  cord.  This  zone  of  hyperaesthesia  and  the  upper 
limit  of  the  anaesthesia  are  valuable  guides  to  the 
position  of  the  lesion. 

Total  loss  of  sensibility  is  often  accompanied  by  the 
formation  of  bedsores  or  crops  of  herpes,  both  of  which 
are  due  to  those  disturbances  in  nutrition  generally 
described  as  "  trophic."  No  impulses  from  the  bladder 
and  rectum  can  reach  the  brain,  nor  can  any  pass  down- 
wards, with  the  result  that  control  over  the  sphincters  is 
lost.  The  bladder  either  discharges  its  contents  reflexly 
at  irregular  intervals,  or,  as  more  frequently  happens, 
it  becomes  distended  until  the  incontinence  of  overflow 
ensues. 

If  the  patient  lives,  the  more  acute  symptoms  pass 
off,  and  the  permanent  effects  of  degeneration  subsequent 
to  the  transverse  lesion  become  apparent.  (Plate  10.) 
The  fibres  of  the  crossed  pyramidal  tracts  degenerate 
downwards,  with  all  the  accompanying  attributes  of 
lateral  sclerosis.  The  legs  become  rigid,  with  or  without 
some  return  of  power  according  to  the  intensity  of 
the  initial  lesion,  and  the  rigidity  may  be  succeeded 
by  contractures,   \n   ^\hich,   in   bad   cases,  the  strength 


PLATE    10.  —  lustantaneous  photograph   showing  gait  in 
'paraplegia  following  myelitis. 


MYELITIS  203 

of  the  flexors  and  adductors  overpowers  that  of  the 
extensors  and  abductors.  The  tendon  reflexes  are 
very  exaggerated,  clonus  appears,  and  the  planlar  reflex 
gives  a  typical  extensor  response.  There  is  no  wasting, 
and  no  electrical  changes  are  present.  Whether  con- 
trol over  the  sphincters  is  entirely  or  only  partially 
regained  depends  upon  the  amount  of  communication 
that  is  re-established  after  the  acute  symptoms  have 
subsided; 

Such  are  the  main  points  to  be  observed  in  a  trans- 
verse myelitis  of  the  lower  dorsal  region. 

2.  A  transverse  lumbar  lesion  destroys  many  motor 
cells  of  the  anterior  horns,  from  w^hich  fibres^go  to  supply 
the  muscles  of  the  legs,  and  hence  there  is  a  considerable 
amount  of  wasting.  The  centres  for  the  bladder  and  the 
reflexes  are  also  liable  to  destruction ;  consequently 
the  superficial  and  deep  reflexes  of  the  legs  disappear, 
and  the  bladder  becomes  a  flaccid  bag  from  which  the 
urine  continually  runs,  i.e.  true  incontinence  as  opposed 
to  the  false  incontinence  of  the  distended  bladder.  As 
in  transverse  myelitis  of  the  dorsal  region,  there  will 
be  anaesthesia  below  the  level  of  the  lesion. 

3.  In  myelitis  of  the  cervical  region  there  will  be 
paralysis  and  anaesthesia  below  the  level  of  the  lesion, 
as  in  the  dorsal  region.  In  addition,  there  is  the  possible 
presence  of  hyperpyrexia  and  of  inequality  of  the  pupils 
(due  to  interference  with  the  cervical  sympathetic  nerves). 
If  the  lesion  chances  to  be  at  the  level  of  the  cervical  en- 
largement, there  will  be  wasting  of  the  muscles  of  the 
arm  from  destruction  of  their  motor  cells,  just  as  the 
muscles  of  the  leg  waste  when  the  lumbar  enlargement 
is  affected.  If,  when  no  intercostal  action  is  possible, 
the  motor  cells  of  the  phrenic  nerves  at  the  level  of  the 
3rd,  4th,  and  5th  cervical  roots  are  destroyed,  death 
takes  place  from  failure  of  respiration. 

4.  Acute  ascending  myelitis,  in  which  the  inflammatory 
process  commences  in  the  lower  parts  of  the  cord  and 
extends  upwards,  sometimes  occurs.  The  chief  symp- 
toms are  rapidly  ascending  paralysis  with  loss  of  sensation, 


204    DISEASES   OF   THE   NERVOUS   SYSTEM 

loss  of  control  over  the  sphincters,  and  the  formation 
of  bedsores.  The  whole  thickness  of  the  cord  is  acutely 
inflamed,  and  its  structure  destroyed. 

5.  Disseminated  acute  myelitis,  in  which  the  inflamed 
areas  are  scattered  throughout  the  cord,  is  sometimes 
found. 

Diagnosis. — In  making  a  diagnosis  it  is  first  neces- 
sary to  be  certain  that  the  lesion  is  in  the  cord,  and  the 
methods  of  ascertaining  this  point  will  be  found  de- 
scribed under  the  heading  of  Paraplegia  (p.  183).  When 
this  is  established  it  is  necessary  to  consider  the  nature  of 
the  lesion.  The  acute  onset  suggests  an  inflammatory 
origin.  Haemorrhage  into  the  substance  of  the  cord, 
besides  being  very  rare,  has  a  more  sudden  onset,  and 
would  be  unlikely  to  assume  a  transverse  distribution, 
though  bleeding  into  a  cavity  of  syringomyelia  might 
in  certain  circumstances  give  rise  to  difficulty. 

The  back  must  always  be  carefully  examined  to  see 
if  the  symptoms  may  possibly  be  due  to  pressure. 

Meningitis  may  simulate  myelitis,  but  in  the  former 
there  are  usually  radiating  pains  due  to  irritation  of  the 
nerve  roots  ;  the  two  conditions  may,  however,  coexist, 
as  so  often  happens  in  syphilitic  disease  of  the  cord. 

An  acute  myelitis  which  ascends  the  cord  will  closely 
resemble  a  case  of  Landry^ s  paralysis,  but  in  the  latter 
there  are  usually  no  less  of  sensation,  no  loss  of  control 
over  the  sphincters,  no  wasting,  and  no  electrical  changes 
in  the  muscles. 

The  Wassermann  reaction  of  the  cerebro-spinal  fluid 
will  assist  in  determining  the  nature  of  the  lesion. 

When  the  patient  is  seen  for  the  first  time  after  the 
acute  stage  has  passed,  the  resulting  degeneration  will 
be  in  evidence. 

The  diagnosis  will  then  chiefly  have  to  be  made  from 
ataxic  jyaraplegia,  subacute  combined  degeneration,  dis- 
seminated sclerosis,  and  tumour  of  the  cord.-  The  decision 
must  dej^end  largely  on  the  history  of  the  onset  and  the 
course  of  the  disease,  since  the  physical  signs  will  often 
be  compatible  with  diseases  of  different  origin. 


MYELITIS  205 

Prognosis. — Myelitis  of  the  cervical  region  of  the 
cord  always  threatens  to  destroy  life  by  its  proximity 
to  the  nuclear  origin  of  the  phrenic  nerves  and  to  the 
structures  of  the  medulla.  When  the  disease  is  situ- 
ated in  the  dorsal  and  lumbar  region  there  is  less 
immediate  danger,  but  if  the  structures  of  the  cord  are 
permanently  destroyed  the  exhaustion  accompanying 
bedsores  and  cystitis  gradually  carries  ofi  the  patient. 
Where  partial  recovery  takes  place  the  patient  may,  with 
care,  live  many  years. 

At  the  beginning  of  a  case  it  is  impossible  to  predict 
with  any  certainty  how  much  power  will  be  regained. 
Some  patients  recover  in  an  extraordinary  way,  but  the 
majority,  after  reaching  a  certain  point,  make  no  more 
progress,  and  for  the  rest  of  their  lives  suffer  from  various 
degrees  of  spastic  paraplegia,  with  or  without  some  diffi- 
culty in  exercising  efficient  control  over  the  sphincters. 

Treatment. — It  is  generally  recommended  that  the 
patient  should  lie  on  his  side  or  stomach,  to  avoid  con- 
gestion of  the  diseased  part,  but  it  is  doubtful  if  position 
really  makes  any  difference.  Attention  must  be  paid 
to  the  state  of  the  bladder,  and  the  urine  should  be 
drawn  off  regularly,  during  which  operation  the  strictest 
aseptic  precautions  must  be  taken  to  prevent  cystitis. 
He  should  lie  on  a  water-bed,  and  the  greatest  care  must 
be  exercised  to  prevent  the  occurrence  of  bedsores.  The 
bowels  should  be  kept  freely  open.  When  there  is  reason 
to  suspect  syphilis  as  the  cause,  appropriate  treatment 
by  salvarsan  or  mercury  should  be  given,  and  the  latter 
drug  also  often  appears  to  be  useful  in  cases  due  to  other 
causes.  Counter-irritation  is  sometimes  applied  to  the 
spine,  but,  as  it  is  of  doubtful  value  and  may  lead  to  the 
formation  of  sores,  it  is  better  omitted.  When  the  acute 
symptoms  subside,  strychnine  is  useful,  but  it  should  not 
be  given  if  much  rigidity  is  present.  Massage,  passive 
movements,  good  food,  and  general  tonic  treatment 
are  indicated  at  this  stage.  Every  effort  must  be  made 
to  prevent  the  limbs  from  assuming  undesirable  positions 
and  undergoing  contractures. 


206    DISEASES   OF  THE  NEKVOUS  SYSTEM 

Diver's  Paralysis  (Caisson  Disease) 
Symptoms  analogous  in  many  ways  to  those  of  acute 
myelitis  are  apt  to  arise  when  a  sudden  change  is  made 
from  a  very  high  to  a  very  low  atmospheric  pressure, 
such  as  may  take  place  in  the  case  of  divers.  The  motor 
symptoms  are  usually  those  of  paraplegia,  which  may 
either  gradually  pass  off  or  be  followed  by  spastic  paralysis 
such  as  occurs  after  myelitis  ;  but  the  disease  may  injure 
all  the  structures  of  the  cord  and  cause  anaesthesia  and 
loss  of  control  over  the  sphincters. 

The  injury  is  supposed  to  be  caused  by  the  rapid 
escape  from  the  tissues  of  air  which  has  been  absorbed 
under  a  high  pressure ;  and  in  order  to  avoid  this 
danger  the  transition  from  high  to  low  pressure  should 
be  gradual. 


CHAPTER   XXTI 

SUBACUTE   COMBINED   DEGENERATION 

In  this  variety  of  combined  sclerosis  tlie  degeneration 
is  liable  to  run  a  somewbat  rapid  course,  and  is  more 
particularly  associated  with  severe  anaemias  or  otber 
toxic  diseases  to  wbicb  allusion  is  made  in  connection 
with  Ataxic  Paraplegia  (p.  237). 

Symptoms. — The  disease  usually  occurs  in  middle- 
aged  people,  and  in  some  instances  pursues  a  fairly 
definite  course  which  can  be  divided  into  stages,  but 
in  the  majority  of  cases  no  sharp  distinctions  can  be 
made. 

In  the  first  and  longest  stage  the  symptoms  are  some- 
what similar  to  those  which  are  described  under  Ataxic 
Paraplegia,  viz.  weakness  and  rigidity,  with  increased 
reflexes,  and  a  variable  amount  of  ataxy.  With  these 
signs  there  are  generally  pronounced  sensations  of  tingling 
and  numbness  and  pains  of  the  lower  limbs,  to  which 
similar  symptoms  in  the  arms  may  be  added. 

In  the  next  stage  there  is  added  a  loss  of  muscular 
and  cutaneous  sensibility,  the  latter  beginning  in  the 
legs  and  extending  upwards  on  to  the  trunk.  The 
spasticity  during  this  period  also  becomes  more  marked 
and  is  accompanied  by  increased  tendon  reflexes,  extensor 
response,  and  the  other  signs  of  an  upper  motor  neurone 
lesion. 

After  a  variable  time  the  patient  may  enter  a  final 
stage,  in  which  the  spasticity  is  rapidly  superseded  by 
flaccidity,  with  absence  of  tendon  reflexes,  wasting  of 
muscles,  and  loss  of  control  over  the  sphincters.     The 

207 


208    DISEASES   OF   THE   NERVOUS   SYSTEM 

extensor  plantar  response  often  remains.  CEdema  of 
the  legs,  bedsores,  and  cystitis  may  now  occur,  fever  is 
present,  and  the  patient  loses  strength  and  usually  dies 
within  a  few  weeks. 

Patliolofiy. — The  cord  shows  degeneration  chiefly 
of  the  lateral  and  posterior  columns,  which  is  secondary 
to  a  more  localized  and  diffused  transverse  lesion  gener- 
ally most  marked  in  the  thoracic  region  of  the  cord. 

Diagnosis. — The  difficulties  in  diagnosis  will  vary 
according  to  the  stages  at  which  the  disease  is  seen. 

When  the  signs  are  those  of  spastic  weakness  and 
ataxy  the  likelihood  of  disseminated  sclerosis  being  present 
has  to  be  considered.  The  main  points  in  which  dis- 
seminated sclerosis  differs  are  in  the  earlier  age  of  onset, 
the  variableness  of  symptoms,  the  tendency  to  optic 
atrophy  and  cranial  nerve  palsies,  and  the  presence  of 
nystagmus. 

As  the  disease  progresses  the  degeneration  may  be 
referred  to  the  results  of  a  previous  transverse  myelitis 
or  accounted  for  by  the  presence  of  a  chronic  myelitis. 

When  the  knee-jerks  are  lost  and  there  is  flaccid 
paralysis,  peripheral  neuritis  has  to  be  thought  of,  but 
the  anaesthesia  of  the  trunk,  the  incontinence  of  urine, 
and  an  extensor  plantar  response  are  against  it,  as  also 
is  the  history  of  the  disease. 

The  loss  of  power,  wasting  and  electrical  changes 
in  the  muscles,  together  with  absence  of  the  Argyll- 
Robertson  pupil,  will  enable  tahes  to  be  excluded. 

Sometimes  the  signs  may  suggest  the  presence  of  a 
tumour,  but  the  pains  are  unlike  those  due  to  pressure 
on  roots,  and  the  paralysis  and  sensory  changes  do  not 
show  the  signs  of  being  due  to  pressure  on  one  half  of 
the  cord  more  than  the  other,  as  often  happens  in  the 
case  of  tumours. 

In  the  earlier  stages  subacute  combined  degeneration 
may  have  to  be  diagnosed  from  hysteria.  In  doubtful 
cases  the  most  reliable  sign  of  organic  disease  is  to  be 
found  in  the  extensor  response  of  the  plantar  reflexes. 
If  this  is  obtained  the  organic  nature  of  the  case  is  clear. 


SUBACUTE    COMBINED    DEGENERATION    209 

Where  there  is  any  uncertainty,  these  reflexes  should  be 
repeatedly  examined,  since  they  do  not  always  respond 
in  the  same  manner. 

Prognosis  and  treatment.  —  Hitherto  all  the 
severe  cases  recorded  seem  to  have  ended  in  death,  nor 
has  their  course  been  materially  modified  by  any  drugs. 
Treatment  must  be  directed  towards  improving  the 
general  health,  which  is  always  bad.  Careful  search 
should  be  instituted  for  any  source  of  infection ;  if 
this  is  found  and  successfully  treated,  the  symptoms 
may  be  ameliorated,  or  even  arrested. 


Q 


CHAPTER   XXIII 

DISSEMINATED   SCLEROSIS 

Etiolociy. — Very  little  is  definitely  known  about  the 
origin  of  disseminated  sclerosis. 

While  there  is  no  exact  age  limit,  the  symptoms 
begin  in  most  cases  between  the  ages  of  15  and  35. 
Children  occasionally  suffer,  but  elderly  people  are  very 
rarely  attacked. 

The  number  of  cases  occurring  in  the  two  sexes  is 
about  equal. 

Pathology. — The  disease,  as  its  name  implies,  is 
characterized  by  the  formation  of  islets  of  connective 
tissue  which  are  scattered  throughout  the  length  of  the 
central  nervous  system  and  also  to  some  extent  in  the 
substance  of  the  peripheral  nerves,  more  especially  in 
those  of  cranial  origin.  The  disease  in  classification 
therefore  belongs  both  to  the  brain  and  the  cord. 

The  white  matter  of  the  brain  and  cord  is  princi- 
pally affected,  it  being  somewhat  less  common  to  meet 
with  symptoms  attributable  to  alterations  in  the  grey 
substance. 

The  patches,  which  are  greyish-white  in  colour  and 
usually  sharply  defined,  vary  very  much  in  size,  some 
being  as  large  as  peas  and  discernible  with  the  naked 
eye,  while  others  are  so  small  that  they  are  only  recog- 
nizable with  a  microscope. 

The  changes  appear  to  begin  in  the  neuroglia  rather 
than  in  the  nerves  fibres,  though  on  this  point  there 
is  some  difference  of  opinion.  In  any  case  the  axis 
cylinders  are  the  last  structures  to  degenerate,  which 

210 


DISSEMINATED    SCLEEOSIS  211 

accounts  for  tlie  fact  that  there  is  seldom  any  secondary 
degeneration  to  be  detected  either  above  or  below  the 
sclerosed  site. 

As  the  symptoms  in  the  earlier  stages  of  the  disease 
are  liable  to  remissions,  it  is  probable  that  the  patches 
are  also  liable  to  variations  before  they  become  per- 
manently established.  These  early  changes  may  be 
due  to  vascular  disturbances  with  possibly  some  passing 
oedema,  and  their  variations  probably  correspond  to  the 
remissions  and  exacerbations  which  clinically  are  so 
characteristic  of  the  disease. 

The  principal  suggestions  which  have  been  put  for- 
w^ard  to  account  for  the  origin  of  the  patches  are  : 

1.  That  they  are  due  to  the  effect  of  toxins. 

2.  That  they  are  the  result  of  some  congenital  abnor- 
mality of  the  nerve  tissue. 

3.  That  they  are  due  to  a  micro-organism. 

In  favour  of  the  first  view,  it  is  pointed  out  that  the 
symptoms  sometimes  appear  to  arise  after  the  infection 
of  specific  fevers.  But  where  a  specific  fever  has  preceded 
the  symptoms,  the  probability  is  that  it  has  only  aggra- 
vated them,  or  perhaps  precipitated  their  occurrence. 
Congenital  changes,  probably  beginning  with  a  prolifera- 
tion of  neuroglial  tissue  after  the  manner  of  that  which 
takes  jDlace  in  syringomyelia,  have  been  suggested  as  the 
cause,  but  there  seems  to  be  no  definite  evidence  to 
support  this  hypothesis. 

Of  late  years  the  view  that  the  disease  depends  upon 
some  infective  organism  has  been  largely  held.  Several 
observers  claim  to  have  found  a  spirochsete  in  the  cerebro- 
spinal fiuid  of  some  cases,  and  there  is  little  doubt  that 
this  view  best  explains  the  variations  and  remissions 
that  so  often  occur  during  the  course  of  the  disease. 

Symptoms. — In  the  form  which  has  generally 
been  considered  characteristic  of  the  disease  the  symp- 
toms are  mainly  weakness,  spasticity,  and  ataxy  of  the 
lower  limbs,  together  with  alterations  in  speech,  tremors, 
nystagmus,  and  optic  atrophy.  (Fig.  56.)  This  combina- 
tion of  signs  makes  up  a  very  striking  picture. 


212    DISEASES   OF   THE   NERVOUS   SYSTEM 


The  patient  walks  with  some  degree  of  difficulty ; 
the  feet  shuffle  on  the  ground  owing  to  the  rigidity 
which  impairs  the  freedom  of  the  joint-movements,  whib 


Staccato  I 
epeech    f 


Nystagmus 
_.  Transient  palsies 
I  Pallor  of  optic 
disc 


fintention 
tremors 


jerk 


_rExaggerated 
I  triceps  jerk. 

( Absent  superficial 
( abdominal  reflexes 

_ ) Sphincter 

"  (  trouble 

__  (  Exaggerated 
" "(  supinator  jerk 


\  Exaggerated 
"  *  J  knee-jerks 


Spastic 
weakness 
and  ataxy 


Exaggerated    ) 
tendo-Achlllis  ,- 


J  Ankle 
I  clonus 


j  Extensor 
""^i  response 


1  Fig.  56.— Scheme  of  Ihe  principal  symptoms  occurring  in  the 
course  of  disseminated  sclerosis. 


PLATE    11— Instantaueons  photograph   showing  tremors 
on  drinking  a  glass  of  water  in  a  case  of  disseminated 

sclerosis. 


DISSEMINATED    SCLEROSIS  213 

there  is  also  an  unsteadiness  of  gait  owing  to  changes 
in  the  posterior  and  cerebellar  tracts. 

When  the  patient  is  seated  there  is  little  to  notice, 
yet  any  movement  of  the  arm  or  hand  is  accompanied 
by  a  coarse  tremor,  which  increases  in  amplitude  as  the 
movement  is  continued.  Thus,  if  the  patient  be  asked 
to  carry  a  glass  of  water  to  his  lips,  the  movements 
become  more  and  more  exaggerated,  until  the  contents 
of  the  glass  are  dashed  to  the  ground.  (Plate  11.)  In 
the  same  way,  if  he  be  asked  to  write,  the  pen  is  drawn 
spasmodically  across  the  paper,  though,  of  course,  in 
the  earlier  stages,  all  grades  of  impairment  of  writing 


7^$A-;/> 


*^{^. 


Fig.  .57.— Specimen  of  writing  in  a  case 
of  disseminated  sclerosis. 

are  encountered.  (Fig.  57.)  In  those  engaged  in  clerical 
work  this  symptom  is  sometimes  the  first  noticed.  This 
tremor,  often  known  as  "  volitional  "  or  "  intentional  " 
tremor,  is  by  no  means  necessarily  confined  to  the  arms. 
Sometimes  there  are  rhythmical  movements  of  the  head, 
which,  however,  cease  when  the  muscles  of  the  neck  are 
supported  on  a  pillow. 

The  tremor  of  disseminated  sclerosis,  then,  is  char- 
acterized by — 

(1)  Its  occurrence  during  voluntary  movements. 

(2)  Its  cessation  during  rest. 

(3)  The  coarseness  of  its  vibrations,  which  in- 

crease  in  amplitude  with  the  continua- 
tion of  the  movement. 
The  power  of  the  legs  (and  probably  of  the  arms)  wiU 
be  found  to  be  considerably  less  than  normal. 

The  knee-jerk  and  tendo-AchilUs  jerk  are  increased, 
ankle-clonus  is  often  obtained,  and  the  plantar  reflex 
shows  the  extensor  response  ;  there  may  also  be  some 
tendency  to  clubbing  of  the  feet. 


214    DISEASES   OF  THE   NEKVOUS   SYSTEM 

In  the  upper  limb  also  the  deep  reflexes  are  generally 
found  to  be  exaggerated,  and  a  brisk  jaw-jerk  will  in  all 
probability  be  obtained,  but  the  superficial  reflexes  of  the 
abdomen  are  frequently  lost. 

As  regards  the  ocular  signs,  nystagmus  is  observed 
when  the  patient  attempts  to  look  to  one  or  the  other 
side,  and  there  may  be  some  weakness  (usually  transient) 
of  one  or  more  muscles,  giving  rise  to  double  vision. 
The  pupils  usually  react  normally  to  light  and  on  accom- 
modation. Changes  in  the  optic  nerves  occur  in  a  large 
proportion  of  cases  of  disseminated  sclerosis,  and  failure 
of  vision  may  be  the  patient's  earliest  complaint.  The 
alteration  most  commonly  seen  is  that  of  pallor  of  the 
optic  disc,  indicating  a  certain  degree  of  atrophy.  In  a 
very  small  percentage  of  cases  signs  of  antecedent 
neuritis  have  been  noted.  The  atrophy  is  often  con- 
fined to  the  temporal  half  of  the  disc,  and  frequently 
affects  only  one  eye.  Only  exceptionally,  therefore,  is 
total  blindness  likely  to  occur.  The  fields  of  vision  may 
show  restricted  perception  of  colours  before  the  sensation 
to  ordinary  light  is  demonstrably  impaired. 

The  speech  is  known  as  "  scanning  "  or  "  staccato," 
from  the  pronounced  manner  in  which  every  syllable  is 
jerked  out. 

There  is  generally  impairment  of  sphincter  control 
at  some  time  during  the  course  of  the  disease. 

Mental  instability  is  often  apparent,  as  is  shown 
by  a  tendency  to  laugh  and  cry  when  there  is  but 
slight  emotional  stimulus ;  occasionally  epileptiform  fits/ 
occur. 

Such  are  the  main  points  to  be  observed  in  a  so-called! 
typical  case,  but  it  by  no  means  follows  that  the  signs 
always  appear  in  this  pronounced  form  ;  indeed,  the 
majority  of  patients  when  first  seen  do  not  present 
a  picture  anything  like  this,  and  may  go  on  for  years 
with  vague  and  transient  symptoms,  the  cause  of  which 
is  often  attributed  to  hysteria  unless  careful  examina- 
tions are  repeatedly  made.  It  therefore  happens  that 
these  so-called  atypical  cases  are  of  the  greatest  import- 


DISSEMINATED    SCLEROSIS  215 

ance,  because  (1)  they  are  more  numerous  than  those 
in  which  signs  are  from  the  first  permanent  and  pro- 
nounced, (2)  they  are  so  apt  to  be  diagnosed  as  functional, 
and  (3)  any  advantage  that  can  be  obtained  from  treat- 
ment is  likely  to  be  far  greater  when  the  changes  in  the 
nervous  tissue  are  capable  of  variation  than  when  they 
have  become  permanent. 

The  patient,  very  commonly  a  girl,  and  usually  some- 
what above  the  age  of  puberty,  may  complain  of  peculiar 
sensations  of  numbness  which  come  and  go  in  a  mysterious 
manner,  and  for  which  no  obvious  cause  can  be  found, 
although  they  sometimes  tend  to  be  more  severe  after 
fatigue  or  at  the  time  of  the  menstrual  period.  Examina- 
tion during  such  an  attack  may  or  may  not  reveal  a 
patch  of  anaesthesia  which  does  not  seem  to  correspond 
to  any  definite  anatomical  arrangement,  and  then  perhaps 
everything  passes  off  for  a  time,  and  all  is  forgotten 
until,  later  on,  one  of  the  legs  drags,  or  all  power  suddenly 
goes  out  of  the  arm,  again  to  return  or  vary  from  time 
to  time.  It  may  be  (and  this  is  very  significant)  that 
there  is  a  temporary  complaint  of  double  vision,  and 
there  is  very  often  transient  difficulty  in  exercising  full 
control  over  the  sphincters. 

In  all  such  cases  the  greatest  care  must  be  taken 
before  organic  disease  can  be  excluded,  and  where  the 
history  is  suspicious  and  the  results  of  examination  are 
negative  it  is  often  wise,  if  possible,  to  postpone  giving  a 
definite  opinion,  for  signs  which  are  absent  at  one  time 
may  frequently  be  elicited  with  ease  at  another. 

Pallor  of  an  optic  nerve,  nystagmus,  squint,  slight 
incoordination  of  the  arms,  and  an  extensor  response 
of  the  plantar  reflexes  are  among  the  most  important 
things  to  note,  and  the  establishment  of  one  sign  which 
is  of  definitely  organic  origin  is  as  useful  in  a  difficult 
case  as  is  the  discovery  of  a  single  tubercle  bacillus  in 
a  case  of  suspected  pulmonary  disease,     i    i  »\-    •    ' 

In  other  cases,  although  the  symptoms  are  obviously 
of  organic  origin,  they  may  for  a  time  be  so  restricted 
as  to  give  rise  to  doubt  regarding  their  nature,  as,  for 


216    DISEASES   OF   THE   NERVOUS   SYSTEM 

example,  when  a  spastic  or  an  ataxic  paraplegia  is  the 
only  condition  present. 

In  yet  others  the  patches  of  sclerosis,  though  widely 
distributed,  may  not  be  in  the  positions  to  produce 
the  typical  signs  of  the  disease,  and  thus  arises  another 
set  of  cases  not  conforming  to  type. 

Diagnosis. — It  will  be  evident  that  the  diagnosis 
of  disseminated  sclerosis  may  be  very  easy  or  the  reverse, 
and  further,  that  the  difficulties  will  vary  during  different 
phases  of  the  disease. 

1.  During  the  early  stages  of  its  development  the 
tendency  is  to  mistake  it  for  hjsteria.  The  chief  points 
to  be  noted  in  making  the  diagnosis  have  already  been 
mentioned. 

2.  When  the  patches  are  situated  exclusively  in  the 
course  of  the  fibres  of  the  lateral  tracts,  primary  lateral 
sclerosis  will  be  thought  of,  and,  in  the  absence  of  any 
further  signs,  time  alone  may  determine  the  diagnosis  ; 
but  it  must  be  remembered  that  primary  lateral  sclerosis 
is  rare,  and  that  many  cases  which  appear  at  first  to 
be  of  this  nature  eventually  develop  signs  of  the  dis- 
seminated type. 

Aid  may  often  be  obtained  from  the  variability 
of  symptoms,  for  primary  lateral  sclerosis  is  a  disease 
of  gradual  but  steady  progress,  whereas  remissions  are 
very  characteristic  of  disseminated  sclerosis. 

When  there  are  patches  in  the  posterior  in  addition 
to  the  lateral  columns,  and  no  symptoms  point  to 
sclerosis  elsewhere,  the  diagnosis  for  a  time  may  be 
ataxic  paraplegia,  or  an  incomplete  transverse  myelitis. 
Ataxic  paraplegia  is,  however,  a  clinical  manifestation 
that  may  depend  upon  various  underlying  pathological 
conditions,  and  it  is  therefore  always  wise,  when  making 
the  diagnosis,  to  keep  in  mind  the  further  developments 
which  so  many  of  these  cases  undergo. 

3.  When  the  signs  are  well  developed,  yet  are  not 
those  which  characterize  the  recognized  types  of  the 
disease,  there  may  be  some  hesitation  in  distinguishing 
the  condition  from  Friedreich's  ataxy,  paralysis  agitans, 


DISSEMINATED    SCLEROSIS  217 

cerebellar  disease,  and,  in  children,  from  cerebral  diplegia. 
General  paralysis  of  the  insane  sometimes  shows  symp- 
toms which  resemble  those  of  disseminated  sclerosis,  but 
the  inequality  of  the  pupils  and  the  loss  of  the  light 
reflexes,  together  with  the  mental  changes  present,  do 
not  usually  leave  the  diagnosis  in  doubt. 

Paralysis  agitans  can  seldom  give  rise  to  real  diffi- 
culty, for  the  tremor  is  finer,  occurs  during  rest,  and  is 
diminished  rather  than  otherwise  on  movement,  while 
rigidity  is  seldom  absent. 

Confusion  with  cerebellar  disease  is  only  likely 
to  arise  in  very  chronic  cases.  In  cerebellar  tumours 
optic  neuritis,  headache,  and  vomiting  are  distinguishing 
features.     Cases  of  cerebellar  ataxia  of  childhood  may 

Fig.  58.— Specimen  of  writing  from  a  case  of  chronic 
poisoning  by  mercury- 

sometimes  resemble  disseminated  sclerosis,  as  also  may 
some  of  the  less  typical  instances  of  cerebral  diplegia. 
A  tremor  which  closely  resembles  that  of  disseminated 
sclerosis  may  sometimes  arise  from  chronic  alcoholism  and 
also  as  a  result  of  chronic  mercurial  poisoning  (Fig.  58), 
but  the  history  of  these  cases,  and  the  ages  at  which 
they  occur,  together  with  the  absence  of  other  signs 
of  disseminated  sclerosis,  generally  make  the  diagnosis 
clear. 

Prognosis  and  treatment. — The  outlook  for 
patients  with  disseminated  sclerosis  is  not  hopeful,  since 
experience  has  shown  that,  despite  the  transient  nature 
of  certain  early  symptoms,  a  temporary  improvement  is 
the  most  that  can  generally  be  expected.  Sometimes, 
however,  there  are  remissions  even  for  years ;  and,  very 
occasionally,  cases  have  been  recorded  in  which  no 
relapse  seems  to  have  taken  place.     When  the  patient 


218    DISEASES   OP  THE   NERVOUS  SYSTEM 

comes  under  treatment  early,  it  is  doubtless  possible  fre- 
quently to  retard  the  course  of  the  disease  by  insisting  on 
the  avoidance  of  fatigue  during  the  stages  of  remission, 
and  on  complete  rest  during  those  of  exacerbation. 

Vaccines  and  inoculations  of  different  kinds  have 
been  recommended  with  little  success,  but  it  is  possible 
that  these  forms  of  treatment  will  be  further  developed 
in  the  future  if,  as  seems  likely,  it  becomes  definitely 
established  that  a  micro-organism  is  the  cause  of  the 
disease.  Urotropin  may  be  tried  on  account  of  its 
supposed  antiseptic  effects  on  the  cerebro -spinal  fluid. 

The  general  nutrition  should  be  carefully  maintained 
by  a  diet  containing  an  abundance  of  milk.  Tonics, 
especially  those  containing  iron,  are  useful;  arsenic 
also  has  gained  some  reputation  in  this  disorder,  but 
no  drug  is  known  to  have  any  specific  effect  upon  the 
lesions.  Massage,  carefully  regulated,  is  of  use  during 
certain  phases,  and  in  the  more  chronic  conditions 
selected  exercises  may  be  tried  with  a  view  of  improving 
the  accuracy  of  the  movements. 


CHAPTER  XXIV 

PROGRESSIVE    MUSCULAR    ATROPHY    AND 
PROGRESSIVE   BULBAR   PARALYSIS 

Progressive  muscular  atrophy  is  the  title  given  to 
those  cases  in  which  wasting  of  muscles  results  from 
a  slow  and  progressive  degeneration  of  the  motor  cells 
in  the  anterior  horns  of  the  spinal  cord,  or  of  their 
analogues  in  the  medulla  and  midbrain. 

In  order,  however,  adequately  to  understand  the 
nature  of  the  disease,  it  is  necessary  to  take  a  rather 
wider  view  of  its  pathology  and  to  regard  it  as  an  affec- 
tion of  the  whole  motor  system  (i.e.  of  the  cell  bodies 
of  both  upper  and  lower  neurones),  in  which  the  lower 
neurones  are  generally  the  more  severely  attacked. 

Etiology. — Most  of  the  cases  occur  towards  middle 
life  ;  more  men  suffer  than  women,  and  heavy  manual 
labour  sometimes  appears  to  be  connected  with  the  onset 
of  the  disease.  In  a  certain  proportion  of  the  cases 
the  degeneration  appears  to  be  due  to  parenchymatous 
syphilis.  In  others  the  cause  is  uncertain,  and  all  that 
can  be  stated  is  that  some  poison,  the  nature  of  which 
is  at  present  unknown,  causes  a  progressive  degenera- 
tion to  take  place  in  the  cell  bodies  of  the  motor 
neurones. 

Symptoms. — The  usual  clinical  picture  of  pro- 
gressive muscular  atrophy  is  due  to  the  predominating 
degeneration  of  the  cells  of  the  lower  motor  neurones, 
those  of  the  upper  neurones  being  generally  affected  to 
a  less  degree;  The  degeneration  of  these  lower  neurones 
does  not  always  begin  at  the  same  level.     Most  often 

219 


220    DISEASES  OF  THE  NERVOUS  SYSTEM 


OCULAR 
NUCLEI 


ARM 
NUCLEI 


the  cells  of  the  cervical  region  of  the  cord  are  the  first 
affected,  and  next  in  frequency  those  of  the  medulla. 
Less  often  the  cells  of  the  lumbar  region  are  attacked 
first,  and  least  often  of  all  the  first  signs  of  the  dis- 
ease arise  from  degeneration  of  the  nuclei  of  the  ocular 

nerves.     (Fig.  59.) 

When  the  disease  begins,  as 
it  generally  does,  in  the  cervical 
region,  the  cells  at  either  the 
upper  or  the  lower  end  of  the 
cervical  enlargement  usually  de- 
generate first,  and  consequently 
the  wasting  is  first  noticed  in  the 
muscles  of  the  shoulder  or  of 
the  hand.  In  the  hand  there  is 
wasting  of  the  interossei  and  of 
the  muscles  of  the  thenar  and 
hypothenar  eminences  (Fig.  60). 
Depressions  appear  between  the 
metacarpals,  and  the  outline  of 
the  first  of  these  bones  becomes 
unduly  prominent  as  the  fibres 
of  the  first  dorsal  interosseous 
muscle  (i.e.  the  abductor  indicis) 
shrivel.  The  fingers  can  no  longer 
be  fully  separated,  and  the  power 
to  abduct  and  oppose  the  thumb 
is  lost.  At  the  same  time  the 
hand  gradually  assumes  a  claw- 
like shape  [main-en-griffe),  and 
the  thumb  tends  to  rotate  so  that 
it  looks  like  a  fifth  finger,  and 
thus  resembles  the  thumb  of  an 
ape.  The  reaction  of  the  wasted 
muscles  to  faradism  and  galvanism  is  diminished,_  and 
later  may  altogether  disappear,  but  there  are  generally 
some  fibres  left  which  still  show  some  reaction  to  fara- 
dism right  up  to  the  end,  and  hence  the  "  reaction  of 
degeneration  "  is  not  usually  present. 


LEG 

nuclei' 


Fig.  59.— Diagram  show- 
ing different  posi- 
tions in  which  pro- 
gressive muscular 
atrophy  may  com- 
mence. 


PROGRESSIVE    MUSCULAR   ATROPHY     221 

The  reflexes  in  the  diseased  areas  disappear  as  their 
arcs  become  broken  by  the  degeneration  of  the  motor  cells. 

As  the  motor  cells  are  alone  affected,  there  is  no 
loss  of  sensation,  nor  is  there  usually  any  pain  beyond 
that  of  aching  and  stiffness,  which  may  well  be  due 
to  the  changes  taking  place  in  the  muscles.  Fibrillary 
tremors  are  very  often  present. 

After  a  time,  perhaps  a  few  months,  wasting  begins 
in  the  opposite  hand,  and  as  degeneration  extends  to 


Fig.  60.— Wasting  of  ihands  in  progressive  muscular  atrophy. 

other  levels  of  the  cord  the  muscles  of  the  shoulder 
and  trunk  waste,  and  the  weakness  of  the  trunk  muscles 
may  imperil  the  adequacy  of  the  respiratory  movements, 
thus  disposing  to  pulmonary  complications. 

In  other  cases  the  disease  spreads  upwards  to  the 
nuclei  of  the  bulb,  giving  rise  to  additional  symptoms 
of  progressive  bulbar  paralysis,  which  is  practi- 
cally a  progressive  muscular  atrophy  in  which  the  nuclei 
of  the  bulb  are  attacked. 

In  the  bulb  the  cells  chiefly  affected  are  those  of  the 
10th,  11th,  and  12th  nuclei ;  hence  there  is  loss  of  power 


222    DISEASES   OF   THE  NERVOUS   SYSTEM 

in  the  lips,  tongue,  pharynx,  and  larynx,  as  described 
in  one  of  the  titles  of  the  disease — progressive  labio- 
cjlosso-fharyngeal  and  laryngeal  paralysis. 

The  degeneration  of  the  hypoglossal  nucleus  accounts 
for  the  tongue  symptoms,  the  affected  accessory  portion 
of  the  ]  1th  and  vagal  nuclei  causing  the  pharyngeal  and 
laryngeal  troubles.  The  one  difficulty  is  to  account  for 
the  affection  of  the  lips  without  assuming  degeneration 
of  the  facial  nucleus,  of  which  there  is  often  no  evidence, 
and  the  solution  appears  to  be  that  those  fibres  which 
supply  the  orbicularis  oris,  though  running  in  the  facial 
trunk,  in  reality  have  their  origin  from  the  upper  end 
of  the  hypoglossal  nucleus,  and  therefore  share  in  the 
degenerative  changes  of  the  latter. 

In  a  typical  example  the  gradual  degenerative 
changes  in  the  hypoglossal  nucleus  first  show  themselves 
by  some  weakness  in  the  more  delicate  movements  of 
the  tongue,  so  that  certain  letters  which  require  a  firm 
muscle  for  their  articulation  cannot  be  clearly  pro- 
nounced. Some  such  letters  are  I,  n,  r,  s,  t.  As  the 
change  progresses,  the  tongue  wastes  and  becomes  more 
and  more  powerless,  until  at  last  it  cannot  be  pushed  out 
or  thrust  into  either  cheek,  and  finally  it  lies  helpless 
on  the  floor  of  the  mouth  with  its  loosely  fitting  mucous 
membrane  wrinkled  over  it.  At  the  same  time  the  lips, 
formed  by  the  orbicularis  oris,  which  derives  its  nerve 
supply  from  the  upper  part  of  the  hypoglossal  nucleus, 
also  show  signs  of  weakness  and  wasting.  At  first, 
certain  letters,  such  as  o,  u,  p,  h,  m,  requiring  the  lips 
to  be  pressed  closely  together  for  their  formation,  are 
blurred,  and  later  the  lips  become  thin,  wasted,  and  so 
weak  that  they  can  no  longer  be  approximated,  the 
saliva  constantly  dribbling  from  the  mouth.  It  is  the 
drooping  of  the  lower  lip  that  helps  so  much  to  give 
the  characteristic  facial  expression  to  these  cases. 

The  degeneration  of  the  10th  and  11th  nuclei  causes 
various  degrees  of  difficulty  in  swallowing.  Fluids 
regurgitate  through  the  nose,  and  the  voice  becomes 
"  nasal  "  owing  to  deficient  movement  of  the  soft  palate 


PROGKESSIVE    MUSCULAR    ATROPHY     223 

incompletely  separating  the  oral  from  the  nasal 
cavity,  while  the  loss  of  the  pharyngeal  reflex  is  a 
source  of  danger  by  allowing  the  food  to  pass  into  the 
larynx. 

When  the  disease  begins  in  the  lumbar  region, 
symptoms  similar  to  those  described  for  the  arms  are 
first  noticed  in  the  feet  and  legs ;  and  in  the  rarer  in- 
stances where  the  nuclei  of  the  3rd,  4:th,  and  6th  cranial 
nerves  degenerate,  a  loss  in  the  corresponding  ocular 
movements  will  be  seen. 

So  far,  we  have  considered  the  eflects  of  the  changes 
in  the  cells  of  the  lower  motor  neurones.  But  while  the 
degeneration  of  the  cells  in  the  anterior  horns  of  the 
cervical  region  of  the  cord  is  proceeding,  some  degenera- 
tion is  frequently  also  taking  place  in  the  fibres  of  the 
lateral  columns  of  the  cord,  as  is  shown  clinically  by 
increased  knee-jerks,  extensor  response  of  the  plantar 
reflex,  and  some  degree  of  weakness  and  spasticity  of 
the  lower  extremities. 

This  degeneration  of  the  lateral  columns  is  secondary 
to  changes  in  the  motor  cells  of  the  upper  neurone  (i.e. 
in  the  cells  of  the  motor  area  of  the  cortex),  to  which 
allusion  has  already  been  made.  The  effects  of  such  upper 
neurone  changes  are,  of  course,  masked  at  levels  where 
advanced  lower  neurone  degenerations  are  present,  but  as 
in  the  majority  of  cases  of  progressive  muscular  atrophy 
the  lower  neurone  changes  are  for  a  considerable  time 
limited  to  the  arms,  the  legs  remain  free  to  show  the  signs 
of  upper  neurone  degeneration.  Hence,  in  cases  of  progres- 
sive muscular  atrophy,  wasting  of  the  arms  is  commonly 
found  combined  with  some  degree — often  very  slight — of 
spasticity  in  the  legs,  together  with  the  corresponding 
changes  in  the  reflexes,  viz.  increased  knee-jerks  and 
extensor  response  of  the  plantar  reflex. 

This  association  of  symptoms  leads  us  a  step  farther, 
for  when  these  spastic  signs  in  the  legs  are  well  marked 
the  condition  is  known  as  amyotrophic  lateral 
sclerosis  (i.e.  a  progressive  muscular  atrophy  of  the 
arms   and   a    lateral  sclerosis  of   the  legs).     No  sharp 


224    DISEASES   OF   THE   NERVOUS   SYSTEM 

distinction  can,  thee,  be  drawn  between  prcgressive  mus- 
cular atrophy  and  amyotrophic  Literal  sclerosis,  for  in 
so  many  cases  of  progressive  muscular  atrophy  there  is, 
in  addition  to  degeneration  of  the  cells  of  the  anterior 
horns,  some  degeneration  of  the  lateral  columns,  and  it 
is  according  to  the  degree  of  this  degeneration  that  the 
case  assumes  the  picture  of  progressive  muscular  atrophy 
or  of  amyotropliic  lateral  sclerosis.  Some  authors  deny 
that  there  is  any  separate  condition  which  should  be 
known  as  amyotro2)hic  lateral  sclerosis,  and  argue  that, 
since  most  cases  of  progressive  muscular  atrophy  show 
some  signs  of  lateral  sclerosis,  the  sejmration  is  an  artifi- 
cial one.  Others,  although  recognizing  the  frequency  of 
the  presence  of  some  degree  of  lateral  sclerosis  in  pro- 
gressive muscular  atrophy,  prefer  to  reserve  the  title 
of  amyotrophic  lateral  sclerosis  for  the  cases  in  which 
the  degeneration  of  the  lateral  columns  is  a  prominent 
and  progressive  feature  of  the  disease.  While  every 
gradation  can  doubtless  be  recognized,  the  two  terms 
are  useful  clinically  to  indicate  whether  the  upper 
neurones  have  or  have  not  undergone  any  material 
degree  of  degeneration.     [See  p.  234.) 

"  Progressive  bulbar  paralysis,"  though  really  relating 
to  a  part  of  progressive  muscular  atrophy,  must  similarly 
be  retained  as  a  useful  clinical  term. 

Diagnosis  of  spinal  forms. — When  the  degenera- 
tion begins  in  the  nuclei  of  the  cord  the  wasting  indicates 
at  once  that  the  disease  is  in  the  lower  neurone.  This 
narrows  the  issue  down  to  lesion  of  the  anterior  horns,  the 
peripheral  nerves,  and  the  muscles  themselves. 

Peripheral  neuritis  must  be  excluded  by  the  absence  of 
pain,  of  anaesthesia,  and  of  the  reaction  of  degeneration, 
and  the  grounds  for  decision  will  be  further  strengthened 
if  the  area  of  wasting  tends  to  correspond  to  the  dis- 
tribution of  motor  roots  and  not  to  that  of  peripheral 
nerves. 

Myopathies. — Some  of  the  myopathies  are  difficult  to 
distinguish  from  progressive  muscular  atrophy,  especially 
when  thev  coirimence  jn  the  muscles  attached  to  the 


PROGRESSIVE    MUSCULAR   ATROPHY     225 

shoulder-girdle,  but  when  they  occur  in  young  people 
about  or  before  the  age  of  20,  or  when  they  attack  the 
facial  muscles  round  the  angle  of  the  mouth,  their  nature 
is  generally  clear. 

Syringomyelia. — Syringomyelia  closely  resembles  pro- 
gressive muscular  atrophy  when  it  commences  with 
wasting  of  the  hand  muscles.  Its  nature  is  demonstrated 
by  the  loss  of  sensation  for  pain  and  temperature,  while 
that  for  touch  is  generally  unimpaired. 

When  the  upper  neurone  is  also  extensively  degener- 
ated the  wasting  in  the  arms  is  often  combined  with 
spastic  paralysis  of  the  legs,  and  the  disease  then  merges 
into  that  of  amyotrophic  lateral  sclerosis,  from  which  it 
can  never  be  very  sharply  distinguished. 

Diagnosis  of  bulbar  iorms. — When  the  degenera- 
tion begins  in  the  nuclei  of  the  medulla,  the  principal 
conditions  it  might  be  confused  with  are  those  arising 
from  diphtheria  and  myasthenia  gravis. 

In  fostdifhtheritic  paralysis  the  symptoms  are  often 
those  of  bulbar  distribution — e.g.  difficulty  in  swal- 
lowing and  regurgitation  of  fluids  through  the  nose — 
but  the  rapid  onset  is  unHke  the  progressive  form.  The 
history  of  an  antecedent  attack  of  diphtheria  makes  the 
diagnosis  plainer,  and  in  the  absence  of  this  the  subse- 
quent history  of  the  postdiphtheritic  paralysis  soon  clears 
the  matter  up.  Exceptionally,  the  effects  of  the  diph- 
theritic poison  are  more  permanent ;  thus,  on  seeing  the 
patient  for  the  first  time,  an  erroneous  diagnosis  might 
be  made,  but  the  non-progressive  character  of  the  lesion 
would  probably  arouse  suspicion  as  to  the  real  nature 
of  the  condition. 

In  myasthenia  gravis  the  symptoms  vary  from  time 
to  time,  and  moreover  there  is  seldom  any  marked 
degree  of  wasting.  The  myasthenic  reaction,  if  present, 
is  diagnostic. 

Vascular  lesions  in  the  bulb  (especially  thrombosis) 

will   cause   paralytic   symptoms   if  they   occur   in   the 

neighbourhood   of  the   nuclei.     Their  acute   onset   and 

more  variable  course  will  serve  as  distinguishing  features. 

p 


226    DISEASES   OF   THE  NERVOUS  SYSTEM 

Acute  inflammation  of  the  bulbar  nuclei  may  occur 
in  cerebral  forms  of  acute  ^poliomyelitis  and  in  cases  of 
lethargic  encephalitis  {see  j).  341). 

Diagnosis  of  the  ocular  forms. — In  those  rare 
instances  where  the  nuclei  of  the  eye  muscles  are  the 
first  to  degenerate,  ocular  palsies  due  to  tabes,  myas- 
thenia gravis,  syphilis,  and  cerebral  tumours  have  to  be 
excluded. 

Prognosis.  —  The  course  which  progressive  mus- 
cular atrophy  usually  runs  is  sufficiently  implied  by  its 
name. 

Extension  to  the  trunk  or  bulb  is  generally  only  a 
matter  of  a  few  years  at  the  most,  and  after  either 
event  the  patient's  life  must  always  be  precarious.  The 
clangers  from  bulbar  paralysis  are  choking  and  broncho- 
pneumonia due  to  particles  of  food  passing  into  the  lungs, 
and  an  attack  of  bronchitis  is  very  apt  to  cause  death 
when  the  trunk  muscles  of  respiration  are  wasted. 

The  prognosis  of  the  parenchymatous  syphilitic 
forms  is  better  than  that  of  other  varieties,  since  there 
is  more  hope  of  the  degenerative  processes  becoming 
arrested.  It  is,  therefore,  important  to  test  the  Wasser- 
mann  reaction  in  all  cases. 

Treatment. — In  the  syphilitic  cases  the  effects  of 
specific  treatment  should  be  tried.  Wliere  the  cause 
is  unknown,  all  that  can  be  done  is  to  endeavour  to 
counteract  its  effects  by  keeping  up  the  nutrition  of  the 
nerves  and  muscles. 

With  regard  to  drugs,  strychnine,  iron,  cod-liver 
oil,  and  other  tonics  are  all  useful.  Strychnine,  given 
regularly,  seems  sometimes  to  check  the  extension  of 
degeneration,  and  it  is  thought  to  be  more  efficient 
when  it  is  given  under  the  skin.  When  there  is  much 
spasticity,  strychnine  should  not  be  given. 

Massage  and  electrical  treatment  in  moderation  help 
to  keep  up  the  strength  of  the  muscles. 

When  bulbar  symptoms  are  present  the  patient  must 
be  fed  carefully  to  prevent  food  from  going  into  the  air- 
passages.     In  bad  cases  a  tube  may  be  required. 


BULBAR    PARALYSIS  227 

Progressive  Muscular  Atrophy  in  Infants 

A  form  of  progressive  muscular  atrophy  which 
occasionally  occurs  in  infants  is  known  as  the  Werdnig- 
Hoilmann  type,  after  the  authors  by  whom  it  was  first 
described.  The  symptoms  closely  resemble  those  of  a 
myopathy,  but  characteristics  of  the  progressive  form 
are  usually  to  be  found  in  the  more  rapid  course  which 
it  runs,  and  in  the  tendency  which  it  shows  to  attack 
the  nuclei  of  the  bulb. 


CHAPTER   XXV 

SPASTIC   PARAPLEGIA;    AMYOTROPHIC    LATERAL 
SCLEROSIS;    ATAXIC   PARAPLEGIA 

SPASTIC  PARAPLEGIA 
Primary  spastic  paraplegia,  or  lateral   sclerosis,  is  an 
example  of  an  uncomplicated  lesion  of  the  upper  motor 
neurones,  and  is  characterized  by  a  degeneration  of  those 
fibres  only  which  compose  the  lateral  tracts. 

Etiology. — This  degeneration  appears  to  be  the 
result  of  nutritional  changes  in  the  motor  cells  in  the 
cerebral  cortex  from  which  the  fibres  arise,  but  to  what 
these  alterations  in  nutrition  are  due  is  uncertain. 
Syphilis,  injury,  lead-poisoning,  and  the  toxins  of  acute 
infective  disease  have  all  been  assigned  as  causes,  and 
from  recent  investigations  it  appears  clear  that  at 
any  rate  a  certain  number  of  cases  are  due  to  neu- 
ronic degeneration  following  parenchymatous  syphilitic 
lesions. 

In  the  majority  of  cases  the  disease  is  first  noticed 
between  the  ages  of  20  and  40. 

Symptoms. — The  main  features  of  this  disease 
are  progressive  Aveakness  and  rigidity,  which  first  show 
themselves  in  the  lower  extremities.  The  patient  notices 
that  he  cannot  walk  so  far  as  he  could  formerly,  and  that, 
after  resting,  the  limbs  feel  stiff  upon  using  them  again  ; 
this  stiffness  is  more  especially  noticed  when  he  first  gets 
up  in  the  morning.  As  the  symptoms  get  worse  he  seeks 
advice,  and  it  is  then  that  the  signs  of  degeneration  of 
the  upper  neurones  are  detected.  Loss  of  power,  rigidity, 
increased  tendon  reflexes,  and  the  extensor  'plantar  response 

228 


SPASTIC    PAKAPLEGIA 


229 


(Fig.  61)  are  present,  but  there  is  no  obvious  wasting, 
and  there  are  no  changes  in  the  electrical  reactions  of 
the  muscles.     There  is  no  loss  of  sensation  or  sphincter 


Exaggerated 
.iaw-jerks 


Exaggerated 
triceps  jerks 


,  Exaggerated 
jsupinatoi*  jerks 


Exaggerated 


JnV       I  I  Exaggerate 

jj\\''\~      (  kuee-jerks 


Weakness  ) 

and 
spasticity 


Exaggerated 

tendo-Achillis  [- 

jerks  ) 


J  Aukle 
( clonus 


J  Extensor 
i response 


Fig.  61.— Scheme  of  the  principal  symptoms  in  spastic  paraplegia. 


230    DISEASES   OT   THE  NERVOUS   SYSTEM 

trouble,  and  there  are,  in  fact,  no  other  signs  to  be  found 
than  those  which  can  be  accounted  for  by  degeneration 
of  the  crossed  pyramidal  tracts. 

As  a  rule  the  symptoms  get  gradually  and  very 
slowly  worse,  until  at  last  walking  becomes  difficult 
or  impossible.  The  rigidity  is  always  a  marked  feature, 
and  sometimes  the  joints,  when  moved  beyond  a 
certain  distance,  spring  into  the  j^osition  of  flexion 
or  extension  from  which  the  term  "  clasp-knife  "  rigidity 
arises. 

The  gait  is  that  known  as  spastic,  and  is  chiefly 
dominated  by  the  rigidity  w^hich  prevents  the  feet  from 
being  freely  raised.  At  the  same  time,  owing  mainly 
to  weakness  of  the  flexors  of  the  ankle-joints,  the  toes 
are  dragged  along  the  ground ;  hence  the  gait  is  stiff, 
and  the  feet  are  shuffled  along  the  ground.  Cramps 
and  spasmodic  twitchings  of  the  legs  are  often  com- 
plained of. 

Diagnosis. — The  diagnosis  of  a  case  of  lateral 
sclerosis  will  be  conducted  somewhat  as  follows : 

The  patient  will  probably  come  complaining  of  loss 
of  power  and  stiffness,  the  existence  of  which  will  be 
confirmed.  On  testing  the  deep  reflexes,  the  knee-jerks 
and  the  tendo-Achillis  jerks  will  be  found  exaggerated, 
as  also  may  be  the  tendon  reflexes  of  the  arm  and  the 
jaw- jerk.  The  plantar  reflexes  will  be  active,  and  the 
big  toe  will  turn  deliberately  upwards  (the  extensor 
response). 

This  combination  of  loss  of  power,  spasticity,  increased 
deep  reflexes,  and  an  extensor  response  warrants  an 
inference  that  the  lateral  columns  are  at  fault,  and 
further  evidence  of  a  negative  kind  in  support  of  this 
inference  will  be  found  in  the  absence  of  wasting  and 
of  electrical  changes  in  the  muscles. 

Signs  may  also  be  present  in  the  arms  showing  that 
the  degeneration  is  going  on  at  that  level.  If  nothing 
else  be  found,  we  are  face  to  face  with  degeneration  of 
the  lateral  columns  only,  and,  always  bearing  in  mind 
the  possibility  of  the  clinical  picture  changing  with  time 


SPASTIC   PARAPLEGIA  231 

into  one  ol  disseminated  sclerosis,  or  of  the  revelation 
of  some  gross  lesion  which  may  for  a  time  be  indiscernible, 
the  diagnosis  of  primary  spastic  paraplegia  may  be 
made.  Primary  lateral  sclerosis  uncomplicated  by  lesions 
of  other  columns  is,  however,  a  rare  condition  ;  indeed, 
some  authors  have  gone  so  far  as  to  doubt  its  existence, 
and  incline  to  the  belief  that  ail  cases  which  at  first 
appear  to  be  of  this  kind  subsequently  develop  other 
symptoms  which  show  that  the  degeneration  is  in  reality 
more  widely  spread.  This  doubt  is  largely  justified 
by  the  scanty  pathological  evidence  that  can  be  brought 
to  show  that  the  degeneration  remains  confined  to  the 
lateral  columns  throughout  the  whole  course  of  the 
disease. 

On  the  other  hand,  degeneration  of  the  fibres  of  the 
lateral  columns  occurs  frequently  in  association  with 
degeneration  of  the  motor  cells  of  the  cord,  giving  rise 
to  the  combination  of  symptoms  known  as  amyotrophic 
lateral  sclerosis,  and  therefore  there  appears  to  be  no 
reason  why  in  certain  cases  the  fibres  of  the  lateral 
columns  should  not  degenerate  independently  of  those 
of  the  lower  neurone,  and  give  rise  to  primary  lateral 
sclerosis. 

In  any  case,  the  greatest  care  must  be  taken  to 
exclude  the  possibility  of  an  extending  lesion  before 
concluding  that  the  disease  is  Hkely  to  remain  a  para- 
plegia due  only  to  primary  degeneration  of  the  fibres  of 
the  lateral  columns. 

A  considerable  number  of  cases,  in  which  at  the  time 
of  examination  the  only  parts  apparently  affected  are 
the  lateral  columns,  prove  later  to  have  been  examples 
of  disseminated  sclerosis  in  the  early  stages,  as  is  shown 
by  the  subsequent  appearance  of  such  signs  as  nystag- 
mus, scanning  speech,  ocular  palsies,  and  loss  of  control 
over  the  sphincters. 

Spastic  paraplegia  may  be  secondary  to  many  con- 
ditions, artiong  which  spinal  caries,  tumours,  myelitis, 
syphilis  of  the  cord,  and  injury  of  the  spine  may  be  men- 
tioned.    In  cases  of  doubt,  the   Wassermann  reaction 


232    DISEASES   OF   THE  NERVOUS  SYSTEM 

of  the  cerebro-spinal  fluid  should  be  tested.  It  may 
happen  that  in  any  of  these  the  spastic  symptoms  are 
for  a  time  sufficiently  predominant  to  make  it  appear 
that  the  lateral  columns  alone  are  diseased.  When,  for 
instance,  in  spinal  caries,  the  cord  is  compressed  by 
inflammatory  and  granulation  tissue,  there  are  often, 
for  a  long  time,  no  well-marked  signs  of  impaired  con- 
duction of  sensory  impulses,  although  the  posterior 
columns  are  presumably  affected.  The  functions  of 
motor  fibres  are  the  more  easily  disturbed,  and  thus 
signs  of  disease  of  the  lateral  columns  appear  first,  and, 
unless  the  back  be  carefully  examined,  the  case  may 
be  passed  as  one  of  primary  spastic  paraplegia. 

Implication  of  the  posterior  columns  {ataxic  'para- 
plegia) will  be  recognized  by  the  presence  of  ataxy 
and  the  other  signs  of  posterior  column  disease  {see 
p.  237). 

Lastly,  spastic  paraplegia  may  be  closely  simu- 
lated by  hysterical  paraplegia. 

In  organic  disease  almost  every  kind  of  symptom 
may  in  its  turn  be  absent  from  any  particular  case,  but 
when  present  such  symptoms  do  not  contradict  one 
another,  as  they  so  frequently  do  in  hysteria.  In  lateral 
sclerosis  the  signs  that  are  present  should  be  compatible 
with  each  other.  For  instance,  the  degree  of  sclerosis 
which  gives  rise  to  an  increase  of  the  knee-jerk  should 
also  give  an  extensor  reaction  of  the  plantar  reflex,  and 
therefore  when,  in  paraplegia,  an  exaggerated  knee-jerk 
occurs  in  company  with  a  permanent  flexor  response  of 
the  plantar  reflex,  it  is  a  contradiction  of  signs  which 
should  at  any  rate  arouse  suspicion.  Ankle-clonus  is 
useful,  when  it  is  well  obtained,  as  a  sign  of  organic 
disease.  In  functional  disease  the  clonus,  when  it  occurs, 
is  generally  feebly  sustained,  and  ceases  when  the  foot 
is  well  pressed  home.  It  must,  however,  be  remembered 
that  this  type  of  clonus,  known  as  pseudoclonus,  often 
occurs  during  different  phases  of  organic  disease. 

In  the  gait  of  functional  disease  the  whole  leg  is 
dragged  after  the  patient  in  a  characteristic  manner,  and 


SPASTIC   PARAPLEGIA  233 

there  are  generally  other  signs,  such  as  anaesthesia,  and 
variations  in  symptoms  which  are  not  compatible  with 
primary  lateral  sclerosis.  Further,  a  sudden  onset  is 
against  primary  lateral  sclerosis,  which  usually  comes 
on  quite  gradually. 

Pathology. — The  characteristic  changes  are  those  pro- 
duced by  a  degeneration  of  the  crossed  pyramidal  tracts, 
but  it  is  rare  to  find  a  spinal  cord  in  which  these  alone  are 
affected.  The  degeneration  probably  begins  in  the  nerve 
fibres  themselves,  and  the  overgrowth  of  neuroglia  is 
secondary.  Probably  the  cause,  as  already  stated,  is 
failing  nutrition  of  the  cortical  cells,  on  which  the  nutri- 
tion of  the  fibres  depends.  There  is  ground  for  believing 
that  the  degeneration  is  in  many  cases  due  to  paren- 
chymatous syphilis. 

Prognosis. — Degeneration  limited  to  the  lateral 
columns  is  not  a  direct  danger  to  life.  The  changes 
progress  slowly,  and  last  over  an  indefinite  number  of 
years.  Sometimes  the  degeneration  appears  to  be 
arrested,  but  there  is  no  possibility  of  cure  in  the  sense 
of  restoration  to  the  previous  condition  of  health. 

Treatment. — Xo  methods  of  ensuring  arrest  of 
the  primary  form  are  known,  but  general  tonics  must 
be  given.  Strychnine  is  often  unsuitable  because  it 
increases  the  rigidity. 

The  general  principles  of  treating  rigidity  (p.  41)  are 
applicable  here. 

In  many  of  the  secondary  forms  the  possibility  of 
removing  the  cause  of  the  degeneration  is,  of  course, 
much  greater. 

Where  the  condition  appears  to  have  a  syphilitic 
origin,  anti-syphilitic  treatment  should  be  given ;  but 
if  the  degeneration  depends  upon  the  parenchymatous 
form,  no  striking  results  are  likely  to  be  obtained. 

\\liere  the  trouble  appears  to  be  due  to  a  tumour 
the  desirability  of  calling  in  the  aid  of  surgery  must  be 
considered. 

In  caries  of  the  spine  (Pott's  disease)  the  pressure 
is  usually  produced,  not  by  the  displaced  vertebrae,  but 


231    DISEASES   OF  THE  NERVOUS  SYSTEM 

by  the  inflammatory  and  granulation  tissue  which  forms 
round  the  cord.  This  adventitious  tissue  may  in 
favourable  circumstances  be  absorbed,  and  treatment  by 
absolute  rest  and  fixation  of  the  spine  over  a  prolonged 
period  (it  may  be  for  one  or  two  years  or  even  more) 
very  frequently  results  in  recovery  of  the  nervous 
symptoms  and  arrest  of  the  disease.  In  more  un- 
favourable cases  an  operation  such  as  transversectomy 
for  evacuating  an  abscess  or  laminectomy  for  relief  of 
pressure  may  be  necessary,  but  the  results  of  laminec- 
tomy in  these  cases  are  not  satisfactory,  and  it  should 
only  be  perforined  when  the  circumstances  seem  specially 
to  require  it. 

Fresh  air,  cod-liver  oil,  iodide  of  iron,  and  other 
methods  of  improving  general  nutrition  are  all  helpful. 

AMYOTROPHIC  LATERAL  SCLEROSIS 

In  amyotrophic  lateral  sclerosis,  degeneration  of 
both  upper  and  lower  motor  neurones  takes  place  at  the 
same  time.  Clinically,  the  symptoms  are  a  combina- 
tion of  those  of  lateral  sclerosis  and  progressive  muscular 
atrophy,  and  reference  has  already  been  made  to  the 
relations  between  amyotrophic  lateral  sclerosis  and  the 
last-named  disease  (p.  223). 

Etiology. — In  some  cases  the  degeneration  is  due 
to  parenchymatous  syphilis ;  in  others  no  definite  cause 
is  known. 

Pathology. — The  essential  cause  of  the  degenera- 
tion is  failure  of  the  nutrition  of  the  motor  cells  of  the 
cerebral  cortex  and  of  the  anterior  horns  of  the  cord. 
Sections  of  the  cord  show  sclerosis  of  the  pyramidal 
tracts  and  wasting  of  the  motor  cells  of  the  anterior 
horns,  while  in  the  brain  some  degeneration  of  the  motor 
colls  of  the  cortex  can  be  demonstrated. 

Symptoms. — The  symptoms  vary  according  to 
the  extent  of  the  wasting  and  spasticity,  but  in  a  typical 
case  the  picture  is  one  of  lateral  sclerosis  together  wdth 
Avasting  of  the  muscles  of  the  upper  limb.  The  onset 
in  such  a  case  is  insidious,  and  usually  resembles  that  of 


AMYOTROPHIC   LATERAL    SCLEROSLS    235 

primary  lateral  sclerosis,  in  which  weakness  and  stiffness 
of  the  legs  are  the  early  signs  ;  but  in  some  cases  wasting 
of  the  hands  is  the  first  sign  to  attract  attention,  and 
then  the  onset  more  closely  resembles  that  of  progressive 
muscular  atrophy. 

The  signs  of  sclerosis  of  the  pyramidal  tracts  are 
generally  very  obvious  in  amyotrophic  lateral  sclerosis 
as  shown  by  the  exaggeration  of  all  the  tendon  reflexes, 
the  occurrence  of  the  plantar  extensor  response,  and  the 
presence  of  clonus,  which  latter  can  often  be  obtained 
in  the  arms  and  jaws  as  well  as  in  the  legs.  (Fig.  62.) 
At  the  same  time  some  wasting  can  be  noticed  to  be 
taking  place  in  the  muscles  of  the  hands  or  arms. 

In  other  cases  the  wasting  is,  at  any  rate  for  a  time, 
the  predominant  feature  ;  the  rigidity  and  weakness 
are  slight,  and  the  clinical  picture  is  then  more  nearly 
like  that  of  progressive  muscular  atrophy  than  that  of 
lateral  sclerosis. 

Diagnosis. — The  diagnosis  of  amyotrophic  lateral 
sclerosis  is  made  from  evidence  of  the  coexistence  of 
sclerosis  of  the  lateral  columns  and  degeneration  of  cells 
of  the  anterior  horns  of  the  cord.  It  thus  forms  a  link 
between  primary  lateral  sclerosis  and  progressive  muscular 
atrophy — and,  indeed,  there  are  few  cases  of  progressive 
muscular  atrophy  in  which  some  signs  of  degeneration 
of  the  lateral  columns  cannot  be  found.  Whether  all 
such  cases  should  be  included  in  the  category  of  amyo- 
trophic lateral  sclerosis  is  largely  a  matter  of  individual 
opinion.  It  is  on  the  whole,  perhaps,  best  to  speak 
of  such  cases  as  progressive  muscular  atrophy  associated 
with  signs  of  commencing  degeneration  of  the  upper 
motor  neurone,  and  to  reserve  the  term  amyotrophic 
lateral  sclerosis  for  those  in  which  the  spasticity  is  a 
predominant  feature  from  the  beginning  ;  but  no  sharp 
line  of  demarcation  can  be  drawn,  since  cases  of  every 
gradation  are  met  with.  When  the  symptoms  arise  in- 
sidiously the  nature  of  the  disease  may  for  a  time  be 
overlooked,  and  the  association  of  the  flabby  muscles 
of  the  arms  with  stiffness  and  weakness  of  the  hands 


236    DISEASES   OF  THE  NERVOUS   SYSTEM 

may  be  attributed  to  rheumatoid  arthritis.  In  consider- 
ing the  diagnosis,  the  possibility  of  syphilitic  origin  should 
not  be  overlooked. 


Exaggerated 
tenion  reflexes  ) 


"^  -/  -  -  -  Jaw-jerk 


Increased 
knee-jerks 


Weakness^ 

and        (  ■  ~  " 
spasticity  ) 


Exaggerated  ) 
tendo-Achillis  \ 
jerks  } 

Extensor 
_  -  -  -   I  response 

Fig.  62.— Scheme  of  the  principal  symptoms  in  amyotrophic  lateral 

sclerosis. 


ATAXIC    PAKAPLEGIA  237 

Prognosis.  —  Amyotrophic  lateral  sclerosis  runs 
much  the  same  course  as  progressive  muscular  atrophy, 
.and  the  dangers  to  life  are  similar  in  both  diseases. 

Treatment.  —  "What  has  already  been  said  of 
progressive  muscular  atrophy  is  applicable  here,  with 
the  exception  that  strychnine  may  have  to  be  with- 
held on  account  of  its  tendency  to  increase  the 
rigidity. 

ATAXIC  PARAPLEGIA 

Ataxic  paraplegia  is  due  to  sclerosis  of  the  lateral 
and  posterior  columns.  As  in  primary  lateral  sclerosis, 
many  of  the  cases  which  at  one  time  appear  to  be  examples 
of  ataxic  paraplegia  develop  other  symptoms  later,  thus 
showing  that  the  degeneration  has  not  been  confined 
to  these  two  columns. 

Etiology. — What  has  been  said  about  the  causation 
of  primary  spastic  paraplegia  is  applicable  here,  but  it 
should  be  added  that  toxins  associated  with  extreme 
anaemias,  diabetes,  and  other  debilitating  diseases  seem 
to  cause  combined  sclerosis  rather  than  one  which 
is  limited  to  the  lateral  columns.  Ergotism  and 
pellagra  may,  among  other  symptoms,  be  accompanied 
by  sclerosis  of  the  posterior  and  lateral  columns  of 
the  cord. 

Symptoms. — The  symptoms  come  on  gradually, 
with  weakness  and  spasticity  of  the  lower  Hmbs,  just 
as  in  lateral  sclerosis,  and  to  these  a  variable  degree 
of  ataxy  is  added.  Thus  the  clinical  picture  is  one  of 
lateral  sclerosis  with  ataxy.  In  some  cases  there  is  a 
difficulty  in  controlling  the  sphincters  ;  in  some,  too, 
sensation  is  blunted. 

Prognosis. — The  disease  runs  a  chronic  course,  and, 
until  the  sclerosis  extends  beyond  the  lateral  and  pos- 
terior columns,  it  does  not  usually  endanger  life. 

Pathology. — The  spinal  cord  shows  sclerosis  of 
the  lateral  and  posterior  columns,  and  also,  in  many 
cases,  of  areas  outside  their  boundaries.  In  the  posterior 
columns  the  posterior  external  column  in  the  neighbour- 


238    DISEASES   OF   THE   NERVOUS   SYSTEM 

hood  of  the  posterior  root  zone  escapes,  in  which  it 
differs  from  the  lesion  of  tabes.  The  reflex  arc  is  thus 
not  broken,  and  the  sclerosis  of  the  lateral  columns 
accounts  for  the  increased  tendon  reflexes. 

Diagnosis. — The  diagnosis  must  generally  be  pro- 
visional in  view  of  the  possible  development  of  other 
symptoms.  As  in  primary  spastic  paraplegia,  a  large 
number  of  cases  prove  later  to  be  examples  of  disseminated 
sclerosis,  as  shown  by  the  occurrence  of  such  symptoms 
as  nystagmus,  ocular  palsies,  and  pallor  of  the  optic  discs. 
In  other  cases  the  symptoms  are  the  beginning  of  the 
subacute  combined  forms  of  degeneration,  as  described 
at  p.  207. 

Degenerative  changes  in  the  cord  after  a  myelitis  may 
cause  symptoms  that  are  practically  indistinguishable 
from  those  of  ataxic  paraplegia. 

Treatment. — No  adequate  method  of  arresting  the 
degeneration  is  known.  Treatment  must  be  directed 
towards  improving  the  general  health  and  correcting 
any  diseased  condition  from  which  the  degenerative 
process  seems  to  arise. 


CHAPTER   XXVI 

FRIEDREICH'S    DISEASE    (HEREDITARY    ATAXY*) 
AND   HEREDITARY   SPASTIC   PARAPLEGIA 

Etiology. — ^Although  Friedreich's  disease  is  known 
also  as  hereditary  ataxy  the  symptoms  are  seldom 
transmitted  directly  from  one  generation  to  another. 
On  the  other  hand,  the  disease  is  often  found  in  several 
members  of  a  family. 

Both  sexes  are  liable  to  sufier.  The  first  symptoms 
may  be  noticed  somewhere  about  the  age  of  puberty,  but 
they  often  begin  at  an  earlier  date.  Occasionally  an 
acute  infective  disease  appears  to  have  been  the  starting- 
point,  but  the  degeneration  can  seldom,  if  ever,  be 
definitely  traced  to  any  obvious  cause,  nor  does  it 
appear  to  depend  on  syphilitic  taint. 

Pathology. — The  main  lesions  comprise  degenera- 
tion and  sclerosis  of  the  posterior,  lateral,  and  direct 
cerebellar  tracts  of  the  cord,  and  sometimes  also  of 
the  antero-lateral  tracts.  The  primary  change  is  pro- 
bably a  degeneration  of  the  nerve  fibres,  to  which  the 
overgrowth  of  connective  tissue  is  secondary. 

Symptoms. — When  the  parents  are  observant,  as 

*  In  some  families,  certain  structures  show  a  special  disj)ositiou 
to  atrophy  prematurely.  The  reason  for  this  is  obscure,  and  can  at 
present  best  be  explained  by  assuming  an  inherent  lack  of  vitality  in 
the  tissues,  rather  than  the  presence  of  an  accidental  toxaemia,  for 
were  the  degeneration  due  to  the  latter  it  would  not,  probably,  so 
frequently  show  itself  in  several  members  of  a  family.  Premature 
degenerations  of  this  kind  occur  in  the  nervous  and  muscular  systems 
as  well  as  in  other  organs  of  the  body,  and  are  included  in  the 
group  to  which  the  title  of  "abiotrophy"  was  appKed  by  Sir 
William  Gowers. 

239 


2iO    DISEASES   OF   THE  NERVOUS   SYSTEM 

they  are  likely  to  be  if  one  child  after  another  is  struck 
down,  a  change  in  the  shape  of  the  foot  is  often  the 
first  warning  that  anything  is  wrong.  In  others,  clumsy 
movements  of  the  legs  or  arms  are  the  earliest  signs. 
This  clumsiness  is  due  to  the  ataxy  resulting  from 
degeneration  of  the  posterior  and  cerebellar  tracts  ;  it 
resembles  that  of  tabes  dorsalis,  and  is  worse  when  the 
eyes  are  closed.  The  legs  are  generally  first  affected, 
and  the  child  stumbles  and  falls  about  ;  later  on,  the 
disorder  extends  to  the  trunk  and  arms. 

Weakness. — The  pyramidal  tracts  are  affected  as 
well  as  the  posterior  columns  ;  hence  some  weakness 
accompanies  the  ataxy,  and  the  main  symptoms  in  the 
legs  are  those  of  ataxic  paraplegia. 

Reflexes. — The  continuity  of  the  arcs  for  the  tendon 
reflexes  are  usually  broken  at  an  early  period  of  the 
disease,  so  that  the  knee-jerks  are  almost  invariably 
lost. 

The  tendo-Achillis  jerk  and  the  deep  reflexes  of  the 
upper  limb  likewise  cannot  be  obtained. 

When  the  plantar  reflex  is  preserved  it  is  often 
"  extensor  "  owing  to  the  degeneration  of  the  crossed 
pyramidal  tracts. 

Deformities. — The  shape  of  the  feet  alters,  and 
assumes  that  known  as  "  pes  cavus,"  in  which  the 
arch  of  the  foot  is  exaggerated  and  the  toes  become 
deformed  as  "  hammer  toes  "  owing  to  the  contraction 
of  the  tendons,  the  extensor  proprius  hallucis  often 
standing  out  very  prominently  (Fig.  63).  A  lateral 
curvature  of  the  spine  often  develops. 

The  eyes. — There  is  usually  a  slow  lateral  nystagmus 
to  be  observed  when  the  eyes  are  turned  to  one  or  other 
side.  There  is  no  Argyll-Robertson  pupil,  and  optic 
atrophy  has  only  rarely  been  observed. 

Nodding  movements  of  the  head  and  a  slurring, 
stuttering  speech  are  both  common. 

Sensation. — In  spite  of  the  extent  to  which  the  pos- 
terior columns  are  sclerosed,  there  is  seldom  any  appre- 
ciable loss  of  sensation,  at  any  rate  until  the  late  stages 


FRIEDEEICH'S   DISEASE:    DIAGNOSIS    241 


of  the  disease.  This  shows  how  widespread  are  the 
paths  through  which  sensory  impulses  are  conducted 
up  the  cord. 

Diagnosis. — A  well-marked  case  can  give  rise  to 
little  difficulty,  as  the  age  at  which  it  begins  and  the 
characteristic  symptoms  are  very  distinctive.  The  con- 
ditions most  likely  to  cause  confusion  are  juvenile  tabes 
and  disseminated  sclerosis.  Tabes  acquired  in  lat^r  life 
can  usually  be  excluded  by  the  history. 

The  main  points  in  which  Friedreich's  disease  differs 
from  tahes  are  set  out  in  the  following  table  : 


Age  of  onset 

-Pains    ... 

Aroyll-Rohertson  pvpil 
Optic  atrophy  ... 

Nystagmus 
Loss  of  power  ... 


Friedreich's 
Disease 


About  or  before 

puberty 
Absent 

Absent 

Absent   (almost 

always) 
Present 
Present 


Locomotor  Ataxy 


Later,  except  in  rare 
cases  of  juvenile  tabes. 

Characteristic  light- 
ning pains. 

Frequent. 

Frequent. 

Absent. 

Not  common  (unless 
quite  local)  till  Jate 
stages  of  disease. 


Disseminated  sclerosis. — ^Disseminated  sclerosis  is  in 
some  cases  differentiated  with  difficulty,  since  many 
of  the  symptoms  are  common  to  both  affections  ;  but 
the  sloAV,  unvarying  progress  of  Friedreich's  disease 
often  contrasts  with  the  variations  met  with  in  dis- 
seminated sclerosis,  especially  during  the  early  stages. 
The  coarser  and  more  irregular  tremor,  the  ocular 
palsies,  and  pallor  of  the  optic  disc  all  help  to  distinguish 
disseminated  sclerosis. 

Hereditary  cerebellar  ataxy. — Hereditary  cerebellar 
ataxy  of  Marie  very  closely  resembles  Friedreich's 
disease  ;  indeed,  it  is  probably  impossible  to  make  a 
definite  diagnosis  in  every  case. 

The  main  points  which  generally  help  to  differentiate 
hereditary  cerebellar  ataxy  are  the  later  stage  of  onset 


242    DISEASES   OF   THE  NERVOUS   SYSTEM 

and  the  preservation  or  increase  of  tendon  reflexes, 
together  with  the  absence  of  spinal  curvature  and  of 
deformities  of  the  feet. 

Variations  from  type. — It  is  plain  that  with  such  a 
wide  distribution  of  degeneration  considerable  deviation 
must  be  occasionally  expected  from  the  type  originally 
described  by  Friedreich.  Where  the  columns  degenerate 
unequally  it  must  sometimes  happen  that  the  symptoms 


Fig.  63.— Pes  cavus,  from  a  case  of  Friedreich's  disease. 

due  to  an  advanced  condition  of  one  predominate  over 
those  due  to  a  slighter  lesion  of  another  tract,  and  this 
especially  occurs  when  the  lateral  columns  degenerate 
before  the  posterior  and  cerebellar.  In  such  cases, 
spasticity  and  increased  reflexes  are  the  more  prominent 
features.  These  patients  generally  com])lain  first  of  de- 
formity of  the  feet,  and,  from  the  youthful  age  and  the 
history  of  the  onset,  the  diagnosis  of  Friedreich's  disease 
naturally  suggests  itself.     Various  other  signs,   e.g.  a 


HEREDITARY   SPASTIC   PARAPLEGIA    243 

certain  degree  of  ataxia,  nystagmus,  and  trophic  ulcers 
of  the  feet,  may  lend  confirmation  to  the  diagnosis, 
though  this  last  symptom  is  not  usual  in  Friedreich's 
disease.  The  chief  difficulty  met  with  is  the  condition  of 
the  knee-jerks,  which  are  exaggerated.  This,  according 
to  most  authorities,  is  absolutely  against  Friedreich's 
disease. 

I  ■  Thus  we  may  be  faced  with  a  set  of  symptoms  which 
closely  resemble  those  of  Friedreich's  disease,  but  yet 
differ  markedly  from  them  in  the  character  of  the  knee- 
jerks,  the  absence  of  which  authors  regard  as  so  import- 
ant a  sign.  Such  cases  may  be  considered  as  "  atypical 
cases  of  Friedreich's  disease,"  yet,  considering  the  fre- 
quency with  which  they  occur,  it  is  more  satisfactory 
to  class  them  separately  as  cases  of  hereditary  spastic 
paraplegia. 

The  term  hereditary  is  somewhat  unsatisfactory 
here  as  in  hereditary  ataxy,  since  this  condition  is 
likewise  familial  rather  than  inherited,  but  it  is  best 
to  retain  it  since  the  term  hereditary  ataxy  remains 
in  use. 

We  have,  then,  in  hereditary  spastic  paraplegia  a 
disease  which  shows  itself  clinically  by  symptoms  that 
appear  chiefly  to  depend  upon  a  premattire  degenera- 
tion of  the  lateral  tracts,  to  which  may  be  superadded 
symptoms  due  to  some  degeneration  of  the  posterior 
columns  and  occasionally  of  other  parts  of  the  nervous 
system. 

There  is  a  close  relationship  between  hereditary 
spastic  paraplegia  and  Friedreich's  disease,  for  the 
degeneration  in  both  depends  on  some  inherent  lack 
of  vitality ;  but  the  former  can  be  distinguished  from 
typical  cases  of  Friedreich's  disease  by  the  predominance 
of  the  spastic  symptoms,  though  occasionally,  as  might 
be  expected,  the  two  conditions  practically  merge  into 
one  another. 

Prognosis. — The  tendency  of  these  spinal  examples 
of  abiotrophy  is  to  progress  slowly,  but  occasionally  the 
cases  seem  to  be  stationary  for  years. 


24  i     DISEASES   OF   THE  NERVOUS   SYSTEM 


The  author's  impression  is  that  the  progress  of  dis- 
ease in  the  hereditary  spastic  paraplegia  type  is  slower 
and  more  likely  to  be  arrested  than  in  the  Friedreich 
type. 

Trcatmeiit. — No  drugs  are  known  to  have  any 
definite  influence  in  checking  the  progress  of  the  de- 
generation, but  much  can  be  done  by  maintaining  the 
general  nutrition  with  such  drugs  as  cod-liver  oil  and 
iron,  and  by  attending  to  the  general  health  in  every 
way.  Under  healthy  conditions  it  is  rational  to  as- 
sume that  the  progress  of  the  disease  is  likely  to  be 
slower,  and  in  some  instances  it  may 
possibly  be  permanently  arrested. 

Locally,  the  condition  of  the  feet 
must  be  considered,  and,  as  already 
mentioned,  in  cases  of  hereditary 
spastic  paraplegia,  where  there  is  fre- 
quently, for  a  time,  no  ataxy,  the  de- 
formity of  the  foot  is  the  patient's 
chief  complaint.  Here  it  is  that  the 
hammer-toes  are  so  troublesome,  for 
they  are  distorted  in  such  a  way  that 
they  are  always  rubbing  and  pressing 
against  the  boot  and  are  apt  to  be- 
come covered  with  very  painful  corns. 
While  a  suitable  boot  is  very  helpful 
in  relieving  this  tendency,  it  is  often  necessary  to 
devise  some  additional  simple  mechanical  appliance. 
When  the  foot  is  flat  on  the  ground,  it  will  be  ob- 
served that  the  toes  are  almost  straight,  and  in  that 
position  would  run  very  slight  risk  of  being  chafed 
by  the  boot ;  but  the  moment  the  foot  is  raised,  up  go 
the  toes.  The  object  of  the  mechanism  illustrated  in 
Fig.  64  is  to  keep  the  toes  as  far  as  possible  in  the 
position  which  they  are  still  able  to  assume  when  the 
naked  foot  is  placed  flat  upon  the  ground.  A  sole  of 
firm  leather  is  made  to  fit  the  under  surface  of  the  foot, 
and  opposite  the  toes  is  a  tape  which  passes  through 
properly  constructed  holes  in  the  leather  sole,  and  thus 


Fig.  64. —  Device 
for  dealing  with 
hammer-toes. 


FRIEDREICH'S   DISEASE  245 

enables  the  toes  to  be  laced  down.  The  tape,  of  course, 
can  be  changed  as  often  as  necessary.  It  is  simple 
and  inexpensive,  and  patients  state  that  they  find  great 
benefit  from  it. 

As  a  rule,  the  progressive  nature  of  the  disease,  the 
number  of  tendons  and  the  amount  of  fascia  involved 
in  the  deformities,  as  well  as  the  likelihood  of  difficulty 
in  getting  the  parts  to  heal  well,  make  these  cases  un- 
favourable for  operation. 


CHAPTER    XXVII 

SYRINGOMYELIA 

Overgrowth  of  neuroglia  and  the  formation  of  cavities 
in  the  spinal  cord  are  the  chief  characteristics  of  syringo- 
myelia. 

Etiology. — The  disease  occurs  in  both  sexes,  and  the 
symptoms  are  generally  first  noticed  in  the  early  years 
of  adult  life. 

Injuries,  inflammations,  and  vascular  lesions  occasion- 
ally seem  to  be  the  starting-point  of  the  disease. 

Pathology.  —  There  are  different  causes  for  the 
formation  of  cavities  in  the  cord.  In  some  cases 
there  seems  to  be  a  congenital  developmental  defect 
in  the  cord  which  results  in  distension  of  the  central 
canal,  the  walls  of  which  are  lined  with  the  usual 
epithelial  cells.  This  condition  is  known  as  one  of 
hydromyelia. 

In  other  cases  the  proliferation  of  neuroglial  tissue 
is  the  primary  process,  and  the  formation  of  cavities  is 
then  secondary  to  degeneration  and  bears  no  relationship 
to  the  central  canal.  It  has  further  been  suggested  that 
the  gliosis  is  due  to  some  irritant  poison,  but  this  at 
present  is  a  matter  of  speculation.  The  cavities,  which 
vary  both  in  shape  and  in  position,  thus  accounting 
for  the  variations  that  are  met  with  in  the  symptoms 
during  life,  are  generally  situated  in  the  grey  matter  near 
the  centre  of  the  cord,  but  they  often  extend  into  the 
anterior  horns,  and  sometimes  into  the  posterior  horns 
also.  The  walls  of  the  cavity  are  generally  formed  by 
the  proliferated  neuroglial  tissue  (Plate  12). 

246 


PLATE    12.  —  Photographs  showing  cavities  (A.  and  B)   and 

gliomatosis  (C)  of  the, spinal  cord  in  syi-ingomyelia. 

(Pierre  Marie) 


SYRINGOMYELIA  247 

Symptoms. — -The  symptoms  are  due  (1)  to 
loss  of  cord  substance,  and  (2)  to  pressure  on  sur- 
rounding parts  by  the  proliferating  neuroglia,  which 
in  some  cases  amounts  to  a  definite  tumour  forma- 
tion. The  symptoms  must,  of  course,  differ  with  the 
position  and  size  of  the  cavity  and  the  degree  of 
gliosis  around  it,  but  as  the  grey  matter  is  especially 
liable  to  be  affected  there  is  a  certain  degree  of  uni- 
formity in  the  main  symptoms  of  any  consecutive 
number  of  cases. 

1.  The  lesions  of  the  grey  matter  interrupt  im- 
pulses of  pain  and  temperature,  hence  there  is  usually 
loss  of  sensation  to  heat,  cold,  and  pain  (Figs.  65,  66), 
while  the  tactile  sensibility  frequently  remains  -unim- 
paired. 

2.  Injury  of  the  motor  cells  of  the  anterior  horns 
occurs  from  extension  of  the  cavity  or  from  pressure  of 
gliomatous  tissue,  and  is  followed  by  a  slow,  progressive 
wasting  of  muscles. 

3.  There  are  also  trophic  lesions  of  the  skin,  but  the 
mechanism  by  which  they  are  produced  is  uncertain. 

The  excavations  are  usually  most  extensive  in  the 
cervical  region  of  the  cord,  so  that  the  wasting  of  muscles 
is  most  prominent  in  the  arms,  while,  at  the  same  time, 
pressure  on  the  lateral  tracts  often  gives  rise  to  some 
degree  of  weakness  in  the  legs  together  with  increase 
of  knee-jerks,  ankle-clonus,  and  extensor  response  of  the 
plantar  reflex. 

The  trophic  disturbances  are  common  (Fig.  67) ;  some 
of  them  probably  arise  in  the  first  place  from  burns 
and  injuries  which  have  not  been  felt.  Spinal  curva- 
tures also  occur. 

Painless  whitlows,  arthropathies  resembling  those  of 
tabes,  vaso-motor  disturbances  of  the  skin  causing 
localized  ansemias  or  hyperaemias,  excessive  or  deficient 
perspiration,  bullous  eruptions,  local  gangrene,  and 
perforating  ulcers,  are  all  met  with.  The  painless  whit- 
lows in  conjunction  with  other  nutritional  disturbances 
of  the  hands  were  first  described  by  Morvan,  but  it  is 


248    DISEASES   OF   THE  NERVOUS  SYSTEM 

generally  considered  that  "  Morvan's  disease  "  is  a  variety 
of  syringomyelia. 

In  some  cases  the  morbid  processes  spread  upwards 


Fig.  60.— Diagram  showing  loss  of  sensation  for  heat  and  cold  in 
a  case  of  syringomyelia. 


to  the  medulla,  and  atrophy  and  weakness,  sometimes 
one-sided,  of  the  tongue,  face,  and  palate  may  occur. 
Nystagmus  is  also  occasionally  seen. 


SYHINGOMYELIA 


249 


If  the  cavity  is  situated  in  the  lower  part  of 
the  cord  the  symptoms  may  be  mainly  confined  to 
the    legs.      Occasionally   a   case   may   be   met   with   in 


Fig.  66.— Diagram  of  the  same  case   as  in   Fig.  6-5,  showing  areas 
over  which  sensations  of  pain  were  lost  or  blunted. 

which  there  has  been  a  sudden  exacerbation  of  symp- 
toms due  to  haemorrhage  having  taken  place  into  a 
cavity. 


250    DISEASES   OF   THE  NERVOUS   SYSTEM 

Diagnosis. — The  wasting  of  the  hands  may  lead  to 
the  diagnosis  of  frocjressive  muscular  atrophy,  unless  the 
sensations  for  pain  and  temperature  are  tested,  when  the 
real  nature  of  the  condition  will  be  apparent. 

Where  sensory  disturbances  are  present  alone,  care 
must  be  taken  not  to  put  them  down  to  hjsleria. 


rig.  67.— Trophic  changes  and  deformities  of  fingers  in  a  case 
of  syringomyelia. 

When  the  cavities  are  mainly  in  the  posterior  horns 
the  symptoms  may  for  a  time  simulate  those  of  loco- 
motor ataxy. 

Profjnosis. — The  tendency  is  for  the  disease  to 
progress  slowly. 

Treatment  can  only  be  directed  towards  keeping 
up  the  nutrition  of  the  wasting  muscles. 


CHAPTER   XXVIII 

SPINA    BIFIDA;    HiEMATOMYELIA 

SPINA  BIFIDA 
Spina  bifida  arises  from  maldevelopment  of  the  laminae 
of  the  vertebrse,  with  which  malformations  of  the  cord 
and  its  membranes  are  frequently  associated. 

Varieties. — The  following  six  varieties  may,  accord- 
ing to  Sir  John  Bland-Sutton,*  be  recognized  : 

(1)  Myelocele,  in  which  the  central  canal  of  the  cord 
is  open  and  from  which  the  cerebro-spinal  fluid  con- 
tinually drains  aw^ay.  This  variety  is  more  common 
in  stillborn  children,  and  those  who  are  born  alive  usually 
do  not  survive  more  than  a  few  days. 

(2)  Syringomyelocele,  in  which  the  central  canal  is 
dilated  and  forms  the  cavity  of  the  protruded  sac. 
This  form  is  exceedingly  rare. 

(3)  Meningomyelocele,  in  which  the  membranes  and 
the  cord  both  protrude,  but  the  cord,  instead  of  being 
dilated,  is  usually  flattened  on  the  posterior  wall  of  the 
cyst.     This  is  the  commonest  form. 

(4)  Meningocele. — In  this  variety  there  is  a  protru- 
sion of  the  membranes  only,  the  cord  remaining  normal. 

(5)  Mashed  Sfina  bifida  (spina  bifida  occulta),  in 
which  there  is  a  deficiency  of  one  or  more  of  the  verte- 
bral arches  which  is  not  associated  with  any  abnormality 
or  protrusion  of  the  cord  or  membranes. 

(6)  Meningocele  sacralis  anterior,  a  rare  variety, 
observed  only  in  girls  and  young  women,  in  which  the 
sac  projects  through  a  cleft  in  the  bodies  of  the  sacral 
vertebrse  and  presents  in  the  pelvis. 

*  "  Tumours,  Innocent  and  Malignant,"  6tli  editiou. 
251 


252    DISEASES   OP   THE  NERVOUS  SYSTEM 

Spina  bifida  is  apt  to  be  associated  with  symptoms 
of  hydrocephalus  and  syringomyelia.  It  also  gives  rise 
to  various  forms  of  talipes,  and  to  nutritional  disturbances 
such  as  perforating  ulcer.  Other  deformities  of  develop- 
mental origin  may  also  coexist. 

Treatment. — In  cases  where  it  is  possible  to  do 
so,  excision  of  the  sac  is  probably  the  best  form  of 
treatment.  Many  cases  are  best  left  untouched  and 
protected  by  a  suitable  shield.  In  simple  meningoceles 
good  results  are  sometimes  obtained  by  the  injection 
of  Morton's  fluid,  which  causes  the  sac  to  contract. 
Morton's  fluid  consists  of;  Iodine  gr.  x,  potassium 
iodide  gr.  xxx,  glycerin  ad  §i.  From  Il^xv  to  3i  is  the 
amount  usually  injected  at  a  time. 

HEMORRHAGE   INTO   THE   SPINAL   CORD 
(HEMATOMYELIA) 

Etiology. — Haemorrhage  into  the  substance  of  the 
cord  may  be  associated  with  injuries  to  the  spine,  or  it 
may  occur  into  an  area  of  the  cord  already  diseased,  e.g. 
in  myelitis,  tumours,  and  syringomyelia. 

A  primary  spinal-cord  haemorrhage  analogous  to 
that  met  with  in  the  brain  is  very  uncommon. 

Symptoms. — The  symptoms  depend  upon  the 
position  and  the  extent  of  the  haemorrhage.  Sudden 
pain  in  the  back  and  paralysis  are  usually  first  met 
with,  and  in  transverse  lesions  there  is  loss  of  sphincter 
control,  together  with  anaesthesia  of  the  parts  below 
the  lesion. 

The  extravasation  of  blood  is  often  confined  to  the 
grey  matter,  and  consequently  the  sensations  for  pain 
and  temperature  may  be  lost  to  a  greater  degree  than 
the  sensation  for  touch. 

The  cells  of  the  anterior  horns  are  also  liable  to  be 
damaged,  with  resulting  muscular  atrophy. 

If  the  patient  recovers,  there  is  a  Ukelihood  of  some 
spastic  paralysis  below  the  level  of  the  lesion,  and  of 
a  permanent  wasting  of  the  muscles  which  are  supplied 
from  the  anterior  horns  at  the  level  of  the  lesion  ;  thus 


HiEMATOMYELIA  253 

wasting  of  the  muscles  of  the  arms  is  very  liable  to  follow 
haemorrhage  in  the  region  of  the  cervical  enlargement. 
On  the  other  hand,  if  the  haemorrhage  is  confined  to  the 
grey  matter,  there  may  be  no  paralysis  below  the  level 
of  the  lesion. 

Pathology. — There  may  be  a  single  extravasation, 
or  numerous  hsemorrhagic  foci  may  be  present,  and  the 
condition  of  the  surrounding  cord  largely  depends  on 
the  existence  or  absence  of  previous  disease.  After 
injuries  there  is  likely  to  be  some  haemorrhage  beneath 
the  membranes  as  well. 

Diagnosis. — The  sudden  onset  of  the  symptoms 
suggests  haemorrhage.  If  the  spine  is  injured  the  signs 
may  be  masked  by  the  presence  of  extensive  meningeal 
haemorrhage.  An  important  guide,  when  it  can  be 
demonstrated,  is  the  loss  of  painful  and  thermic  sensa- 
tions, w^hile  the  tactile  sense  is  retained,  thus  pointing 
to  a  lesion  of  the  grey  matter.  The  existence  of  previous 
disease  in  the  cord  must  be  decided  by  the  history. 

Prognosis. — The  prognosis  depends  on  the  cause 
and  the  extent  of  the  haemorrhage.  In  cases  of  injury 
where  the  haemorrhage  has  been  slight,  patients  often 
do  well,  although  they  may  have  permanent  paralysis 
if  cells  with  axons  innervating  important  muscles  be 
damaged. 

Treatment. — The  patient  should  be  kept  as  quiet 
as  possible  ;  an  ice-bag  should  be  appHed  to  the  spine. 
The  bowels  should  be  opened.  If  necessary,  morphia 
may  be  given  to  relieve  pain.  Ergot  has  been  recom- 
mended on  account  of  its  supposed  power  of  checking 
the  haemorrhage. 


CHAPTER   XXIX 

TUMOURS   OF    THE    SPINAL    CORD 

Tumours  of  the  spinal  cord  may  be  divided  into  two 
classes,  according  as  they  arise  within  or  without  the 
substance  of  the  cord. 

The  chief  tumours  that  arise  within  the  substance  of 
the  cord  (intramedullary  tumours)  are  gummata,  sar- 
comata, gliomata,  and  tuberculous  growths.  Probably 
the  commonest  of  these  intramedullary  tumours  are 
gummata. 

Gliomata  do  not  occur  so  often  in  the  cord  as  in  the 
brain,  and  intramedullary  tuberculomata  are  decidedly 
uncommon. 

Outside  the  cord  (extramedullary  tumours),  sarco- 
mata, carcinomata,  psammomata,  lipomata,  fibromata, 
gummata,  and  hydatids  are  found ;  and  occasionally 
osteomata  have  been  observed  to  press  upon  the  cord. 
Carcinomata  occur  chiefly  in  the  vertebrae,  and  are  in 
most  cases  secondary  to  disease  elsewhere. 

Sarcomata  generally  arise  from  the  pia  mater,  and 
are  sometimes  widely  diffused  over  the  membranes  of 
the  brain  as  well  as  of  the  cord.  (Plate  13.)  Their 
place  of  origin  is  then  often  difficult  to  determine,  the 
disease  at  times  appearing  to  have  spread  upwards  from 
cord  to  brain,  and  at  other  times  from  brain  to  cord. 
In  the  latter  case  the  extension  may  be  due  to  infection 
of  the  cerebro-spinal  fluid  by  a  primary  growth  situated 
high  up  in  the  nervous  system  and  exposed  to  the  stream 
of  cerebro-spinal  fluid.     (Stanley  Barnes.) 

Psammomata  (dural  endotheliomata)  also  grow  from 
the  pia  mater.     (Fig.  68.) 

254 


6 


tt)  ~ 


\1 


mmmmmmmmimmmmm 


o  -s 


<U     o 


"3    Q 


<  s 


8. 


TUMOURS    OF    THE    CORD 


255 


Lipomata  usually  grow  from  the  areolar  tissue  outside 
the  dura  mater ;  they  may  also  arise  on  its  inner  surface. 

Fibromata  sometimes  arise  in  connection  with  nerve 
roots;  they  are  referred  to  in  Chapter  XV.  (p.  162). 

Gummata  of  the  mem- 
branes are  often  ill-defined 
tumours,  and  are  frequently 
accompanied  by  extensive 
meningeal  infiltration  and 
endarteritis. 

Hydatids  are  important 
causes  of  compression  of 
the  cord.  They  nearly  all 
grow  in  the  areolar  tissue 
between  the  dura  mater 
and  the  bone.  Of  44  cases 
tabulated  by  Scblesinger 
(quoted  by  Bland-Sutton), 
39  were  extradural  and 
only  5  intradural. 

Symptoms . —  The 
symptoms  difier  somewhat 
according  as  the  tumour  is 
situated  outside  or  inside 
the  substance  of  the  cord. 

When  outside  the  cord 
(extramedidlary)  a    tumour       / 

produces  symptoms  by  pres-  Fig.  68.— Portion  of  the  spinal  cord 
sure  (1)  on  the  nerve  roots 
and  (2)  on  the  cord  itself. 

The  nerve  roots  gener- 
ally suffer  first,  so  that  the 
patient  complains  of  fain, 
which  tends  to  follow  the  root  distribution,  and  which 
for  a  time  is  generally  unilateral.  As  the  tumour  grows, 
it  presses  on  the  cord,  and  also  perhaps  on  the  anterior 
nerve  roots,  and  thus  to  the  pain  are  added  iveahness 
and  loss  of  sensation,  which  in  the  early  stages  may 
be  of  the  Brown-Sequard  distribution. 


with  a  psammoma  situated  at 
the  level  of  the  interverte- 
hral  disc  between  the  10th 
and  nth  thoracic  vertebrae. 
From  a  woman  46  years  of  age. 
{Sir  J.  Bland-Sutton ;  Miiaewn, 
Middlesex  Hospital.) 


256    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  weakness  below  the  level  of  the  tumour  will 
be  of  upper  neurone  type  (spasticity,  increased  knee-jerks, 
extensor  response  of  the  plantar  reflex,  with  no  wasting 
or  changes  in  the  electrical  reactions  of  muscles),  while, 
at  the  level  of  the  lesion,  compression  of  one  or  more 
anterior  roots  may  cause  some  paralysis  and  wasting 
over  the  area  of  their  distribution. 

The  main  alterations  in  sensation  are  anaesthesia 
over  the  areas  supplied  by  the  compressed  posterior 
roots,  to  which  may  be  added  subsequently  a  more 
generalized  anaesthesia  below  the  lesion,  due  to  pressure 
of  the  growth  on  the  sensory  columns  of  the  cord.  As  a 
rule,  the  loss  is  more  or  less  equal  for  all  kinds  of  sensa- 
tion. At  the  level  of  the  tumour  there  may  be  a  zone 
of  hyperaesthesia.  With  increasing  compression  a  com- 
plete paraplegia  develops  with  loss  of  power  over  the 
sphincters. 

When  the  tumour  grows  in  the  substance  of  the 
cord  {intramedullary)  there  is  usually  less  pain,  while 
paralysis  and  anaesthesia  are  more  likely  to  be  early 
symptoms. 

The  distribution  of  the  weakness  and  of  the  alteration 
in  sensation  during  the  early  stages  of  an  intramedullary 
growth  depends  upon  the  locality  of  the  cord  in  which 
the  new  growth  originated.  If,  for  instance,  the  sub- 
stance of  the  grey  commissure  is  destroyed,  the  signs 
will  at  first  very  probably  simulate  those  of  syringo- 
myelia, viz.  loss  of  thermal  and  painful  sensibility  with 
preservation  of  tactile  sensation.  To  these  signs  may 
be  added  some  muscular  wasting,  as  the  pressure  is  felt 
by  the  cells  in  the  anterior  horns. 

On  the  other  hand,  if  the  tumour  grows  for  a  time 
in  one  half  of  the  cord,  the  signs  will  tend  towards 
those  of  the  Brown-Sequard  form  of  paralysis,  viz.  loss 
of  power  below  and  on  the  same  side  as  the  tumour, 
with  loss  of  sensation  chiefly  on  the  opposite  side 
{see  p.  21). 

As  the  growth  invades  more  and  more  of  the  cord, 
these  early  distinctions  become  masked  bv  the  onset  of 


TUMOURS    OF    THE    CORD  257 

more  generalized  paraplegia  associated  with  a  variable 
amount  of  anaesthesia  below  the  lesion. 

The  symptoms  are  then  usually  those  of  a  transverse 
lesion  of  the  cord,  viz.  spastic  paralysis,  anaesthesia, 
and  loss  of  power  over  the  sphincters  below  the  lesion. 

Tumours  of  the  cauda  equina. — The  symptoms  of 
tumours  of  the  cauda  equina,  since  the  paresis  is  of  the 
lower  neurone  type,  are  mainly  those  of  flaccid  paralysis 
with  some  wasting  and  electrical  changes.  The  efiect  on 
the  different  structures  varies  with  the  level  of  the  lesion. 
Thus,  the  knee-jerks  may  be  retained  if  the  fibres  going 
to  the  anterior  crural  nerve  are  unaffected ;  the  degree 
of  sphincter  control,  too,  varies  in  different  cases. 

Compression  of  the  cord  from  tumours,  especially  at 
the  lumbar  region  and  cauda  equina,  may  cause  changes 
in  the  cerebro-spinal  fluid  known  as  Froin's  syndrome, 
the  chief  characteristics  of  which  are  yellowish  dis- 
coloration, heavy  coagulation,  and  an  increase  in  the 
number  of  lymphocytes.  Discoloration  of  the  fluid 
only  is  not  pathognomonic  of  compression,  as  it  may 
arise  as  the  result  of  an  old  haemorrhage. 

Diagnosis. — The  recognition  of  tumours  at  an  early 
stage  is  very  difficult,  for  the  cause  of  the  pain  is  often 
indefinite,  and,  in  the  absence  of  other  signs,  is  liable  to 
be  attributed  to  such  conditions  as  muscular  rheumatism, 
neuritis,  and  sciatica.  Suspicion  should,  however,  be 
aroused  by  the  constancy  of  the  pain,  its  tendency  to 
follow  an  area  of  root  distribution,  and  especially  by  the 
'■  girdle  "  character  which  it  often  assumes.  This  suspi- 
cion will  be  confirmed  by  the  discovery  of  progressive 
weakness  and  sensory  disturbance.  When  slow-growing 
cases  of  intramedullary  gro\vth  are  not  accompanied  by 
pain  they  may  be  difficult  to  distinguish  from  degenera- 
tive processes  in  the  cord. 

The  diagnosis  of  a  spinal  tumour  having  been  made, 
it  is  necessary  to  try  to  decide  whether  it  is  situated 
within  or  without  the  cord. 

In  some  cases  the   early  implication   of   the   roots, 
followed    subsequently   by   signs    of    gradual    compres- 
R 


258    DISEASES   OF   THE   NERVOUS   SYSTEM 

sion  of  the  cord,  are  almost  certain  indications  that  the 
growth  is  extrameduUary ;  in  others,  the  dissociated 
anaesthesia,  together  with  the  absence  of  signs  of  pressure 


12th  dorsal 


2nd  lumbar' 

4th  lumbar 

I 
Sacral -^3—;- 


Coccygeal 


2ud  cervical 

H 

■ 

4th  cervical 

^p 

■ 

6th  cervical 

^p 

5 

8tli  cervical 

■HB 

2nd  dorsal 

RIh 

4th  dorsal 

Hi 

6lh  dorsal 

Bm 

8th  dorsal 

ni 

loth  dorsal 

H 

■ 

i 


3rd  cervical 
5th  cervical 
7th  cervical 
1st  doi'sal 

3rd  dorsal 
5th  dorsal 
7th  dorsal 
9th  dorsal 
11th  dorsal 

1st  lumbar 

3rd  lumbar 
5th  lumbar 


Fig.  69.— Diagram  showing  relations  of  the  root  origins  of  the 
spinal  nerves  to  the  spines  of  the  vertebra?.  (After  H.  M  .  Reid, 
"  Qvain's  Anatomy.") 


TUMOUKS    OF    THE    CORD  259 

on  the  roots,  makes  it  almost  equally  certain  that  the 
tumour  is  growing  in  the  substance  of  the  cord.  But 
between  these  two  groups  there  are  many  cases  in 
which  diagnosis  is  difficult,  especially  if  the  patient  has 
already  passed  the  early  stages  of  the  disease. 

The  next  point  to  decide  is  the  level  at  which  the 
tumour  is  situated.  The  guide  to  this  is  to  be  found 
in  the  upper  limits  of  the  signs.  When  the  anaesthesia 
and  pain  are  due  to  pressure  on  roots  their  areas  must 
be  very  carefully  mapped  out  and  identified  with  their 
particular  roots,  and  the  uppermost  nerve  root  that 
is  affected  should  demarcate  the  upper  level  of  the 
tumour.  Any  local  loss  of  power  due  to  pressure  on 
anterior  roots  can  be  utilized  in  a  similar  manner  for 
localizing  purposes. 

When  the  anaesthesia  is  of  intramedullary  origin, 
its  upper  level  will  likewise  denote  the  position  of  the 
tumour,  but  any  zone  of  hypersesthesia  that  may  exist 
about  the  level  of  the  lesion  must  be  taken  into  con- 
sideration, since  its  presence  will  indicate  root  irritation, 
and  will  show  the  necessity  of  locating  the  growth  rather 
higher  than  the  anaesthesia  indicates. 

When  the  sensory  signs  are  taken  to  indicate  the 
level  of  the  lesion,  it  must  be  remembered  (1)  that  the 
origin  of  the  roots  from  the  cord  is  not  level  with  their 
foramina  of  exit,  and  (2)  that  the  spines  of  the  vertebrae, 
owing  to  their  obliquity,  are  not  on  a  level  with  their 
corresponding  bodies.  Unless  these  points  are  kept 
in  mind  there  will  be  a  likelihood  of  the  localization 
being  made  too  low.  The  relations  of  the  nerve  roots 
to  the  vertebral  spines  are  shown  in  Fig.  69. 

Treatment. — If  the  tumour  is  syphilitic,  or  if 
there  is  a  reasonable  doubt  regarding  its  nature,  an 
efficient  course  of  antisyphilitic  treatment  should  be 
tried.  If  the  symptoms  do  not  abate,  the  question  of 
the  removal  of  the  neoplasm  should  be  considered  with- 
out delay.  The  chances  of  success  in  an  operation  for 
this  purpose  are  naturally  greatest  in  the  case  of  extra- 
medullary  growths.     It  is,  however,  also  found  possible 


260    DISEASES   OF  THE  NERVOUS   SYSTEM      ] 

to  deal  to  some  extent  with  cases  of  intramedullary 
origin,  more  especially  by  the  method  of  "  extrusion," 
which  consists  in  making  an  incision  down  to  the  growth 
and  then  allowing  the  tumour  to  be  gradually  extruded 
from  the  substance  of  the  cord  by  the  force  of  the  intra- 
medullary pressure.* 

*  This  method  is   described  in   detail  by  Elsberg  iu  the  Tran>s. 
Seventeenth  International  Congress  of  Jledici)ie,  1913. 


CHAPTER   XXX 

SPINAL   MENINGITIS   AND   MENINGEAL 
HEMORRHAGE 

SPINAL  MENINGITIS 

For  purposes  of  classification,  the  cases  of  spinal  menin- 
gitis may  be  divided  into  inflammations  of  the  dura  mater 
(pachymeningitis)  and  of  the  pia  mater  (leptomeningitis), 
although  it  necessarily  often  happens  that  no  distinction 
can  be  drawn  between  them  either  at  the  bedside  or 
in  the  post-mortem  room. 

Pachymeningitis. — Inflammation  of  the  dura  mater 
may  commence  on  the  inner  or  outer  surface,  and  the 
commonest  causes  are  syphilis,  injury,  and  extension 
of  inflammatcry  processes  from  neighbouring  parts,  e.g. 
from  tuberculous  disease  of  the  vertebrae. 

One  form  {'pachymeningitis  hypertrophica),  in  which 
the  dura  mater  is  greatly  thickened,  is  especially  apt 
to  occur  in  the  cervical  region  of  the  cord,  and  to  cause 
wasting  and  pains  in  the  arms. 

Leptomeningitis. — In  leptomeningitis  the  inflammation 
is  at  first  seated  in  the  pia  mater  and  arachnoid. 

Acute  infections,  as  from  tubercle,  pneumonia,  septi- 
caemia, and  epidemic  cerebro-spinal  meningitis,  are  the 
chief  causes  of  this  variety. 

A  local  serous  meningitis  in  which  a  collection  of 
fluid  in  the  subarachnoid  often  presses  on  the  cord  and 
resembles  a  tumour  may  also  occur. 

Symptoms. — The  symptoms  of  spinal  meningitis 
arise  mainly  from  compression  and  irritation  of  the 
anterior  and  posterior  nerve  roots  and  also  from  exten- 

261 


262    DISEASES   OF   THE   NERVOUS   SYSTEM 

sion  of  the  inflammi-lion  to  the  structures  of  the  corxl. 
Hence  the  symptoms  will  differ  with  the  position  and 
intensity  of  the  process.  In  hypertrophic  meningitis  of 
the  cervical  region,  to  which  allusion  has  already  been 
made,  there  is  generally  a  slow  strangulation  of  the 
nerve  roots  of  the  arms  with  consequent  pains  and 
wasting  ;  and  perhaps  at  a  later  date  there  may  be 
signs  of  pressure  on  the  cord  giving  rise  to  the  effects 
of  an  incomplete  transverse  lesion. 

In  acute  leptomeningitis  the  signs  of  a  general  infection, 
such  as  fever,  rapid  pulse,  and  perhaps  rigors,  are  also 
usually  present.  The  symptoms  of  irritation  of  the 
nerve  roots  are  very  prominent,  and  are  shown  by  pain 
over  the  spine  which  radiates  down  the  limbs,  together 
with  spasms,  cramps,  and  rigidity  of  the  muscles.  The 
head  is  often  drawn  back,  the  back  arched,  and  the 
abdomen  retracted. 

The  control  over  the  sphincters  is  frequently  im- 
paired, and  there  may  be  other  signs  that  the  inflam- 
mation is  extending  into  the  substance  of  the  cord. 
Bedsores  and  crops  of  herpes  are  likely  to  occur. 

In  the  moie  chronic  forms,  pain  is  generally  a  pro- 
minent feature  and  the  motor  symptoms  are  less  severe. 

In  many  of  the  infective  cases  cerebral  meningitis 
is  also  present,  and  to  a  considerable  degree  masks  the 
spinal  symptoms. 

The  principal  characters  of  syphilitic  meningitis  are 
described  in  Chapter  XXXI. 

Diagnosis. — In  its  acute  forms,  spinal  meningitis 
has  to  be  distinguished  from  myelitis. 

Reliance  must  be  placed  chiefly  on  the  signs  of  irrita- 
tion of  the  nerve  roots  which  are  so  typical  of  meningitis, 
while  paralysis  is  likely  to  be  more  marked  in  a  lesion 
of  the  cord.  Of  course  the  two  conditions  may  be  present 
at  the  same  time. 

In  severe  cases  the  symptoms  may  be  suggestive 
of  tetanus,  but  they  are  not  in  keeping  with  the  onset 
of  tetanus,  which  usually  begins  in  the  muscles  of  tie 
jaw  and  neck. 


SPINAL    MENINGITIS  263 

The  symptoms  of  hcBmorrhage  into  the  meninges  are 
more  rapid  in  their  onset. 

In  the  chronic  cases  the  wasting  of  muscles  may 
resemble  that  of  progressive  muscular  atrophy,  but 
the  presence  of  the  pains  and  anaesthesia  generally 
shows  that  the  disease  is  not  confined  to  the  motor 
system.  In  other  instances,  especially  in  the  localized 
serous  variety,  there  is  difficulty  in  distinguishing 
meningitis  from  tumour,  which  the  pressure  symptoms 
often  closely  resemble. 

Examination  of  the  fluid  obtained  by  lumbar  punc- 
ture may  be  an  important  aid  to  diagnosis,  especially 
in  the  acute  cases. 

Prognosis. — The  prognosis  of  acute  spinal  menin- 
gitis is  not  good,  for  the  dangers  of  extension  to  the 
region  of  the  medulla  and  brain  and  to  the  substance  of 
the  cord  are  all  great.  In  a  certain  number  of  cases 
the  symptoms  subside,  leaving  behind  them  a  variable 
amount  of  permanent  damage,  and  in  a  few  instances 
a  good  recovery  takes  place.  In  the  chronic  cases  the 
outlook  varies  with  the  position  and  cause  of  the  in- 
flammation. Where  the  origin  of  the  disease  is  outside 
the  dura  mater,  as  it  is  in  spinal  caries,  the  results  are 
often  satisfactory  if  the  original  focus  of  disease  be 
removed.  Some  of  the  syphihtic  cases,  of  course,  do 
very  well,  but,  as  mentioned  elsewhere  (p.  274),  per- 
manent cure  even  in  these  cannot  be  relied  upon. 

Persistence  or  return  of  symptoms  during  treat- 
ment and  extension  of  the  disease  are  bad  signs. 

Treatment. — Whenever  syphilis  is  suspected,  sal- 
varsan  or  mercury  should  be  administered.  Potassium 
iodide  may  be  given  in  full  doses  at  the  same  time. 
Iodide  is  also  undoubtedly  sometimes  useful  in  cases 
which  apparently  are  not  of  syphilitic  origin. 

When  the  disease  is  chronic  and  has  begun  outside 
the  dura  mater,  the  pressure  on  the  cord  may  sometimes 
be  relieved  by  performing  laminectomy. 

In  the  acute  infective  cases  an  ice-bag  may  be 
applied  to  the  spine,  though  in  many  cases  warm  applica- 


26 1    DISEASES   0^  THE  NERVOUS  SYSTEM 

tions  are  m jre  comfoiiing.  Count cr-inilation  is  useful, 
but  care  must  be  taken  not  to  set  up  bedsores,  especially 
if  myelitis  is  present  at  the  same  time. 

Mercurial  inunction  may  be  used,  as  in  myelitis,  for, 
even  though  there  is  no  history  of  syphilis,  mercury 
seems  often  to  exercise  a  beneficial  influence  over  the 
inflammatory  processes.  Morphia  must  be  given  if 
required. 

The  same  care  in  nursing  and  attention  to  the  bladder 
are  necessary  as  in  myelitis. 

MENINGEAL   HEMORRHAGE 

Etiology. — Haemorrhage  into  the  membranes  of 
(he  cord  may  be  associated  with  haemorrhage  into  the 
brain,  and  is  then  usually  of  secondary  consequence. 
Injury  of  the  spine  is  sometimes  accompanied  by  some 
liaemorrhage,  which  is  more  often  outside  the  dura  mater. 
Acute  fevers,  degenerated  vessels,  and  severe  anaemias 
,are  occasionally  the  causes  of  haemorrhages  into  the 
membranes  of  the  cord  as  elsewhere. 

Symptoins. — The  symptoms  are  those  of  irritation 
( f  nerve  roots  and  pressure  on  the  cord  ;  hence  they 
ere  much  the  same  as  those  of  an  acute  spinal  meningitis, 
except  that  they  arise  more  suddenly. 

There  is  severe  pain  in  the  back  and  along  the  course 
cf  the  nerve  roots,  accompanied  by  a  variable  degree  of 
spasm  of  muscles,  and,  when  the  cord  is  compressed, 
Ihere  is  a  more  or  less  complete  paralysis  below  the 
lesion.  The  deep  reflexes  are  at  first  diminished,  but 
1  iter  become  exaggerated,  and  efficient  control  over  the 
sphincters  is  often  lost. 

Prognosis. — The  prognosis  depends  in  the  first 
ylice  on  the  amount  of  blood  extra vasated  and  on  the 
cfTects  which  it  produces  on  the  cord.  The  extent  of 
ll.e  inflammatory  and  other  after-effects  remains  to  be 
seen,  so  that  in  every  case  the  prognosis  must  necessarily 
be  one  of  caution. 

Diagnosis. — Haemorrhage  into  the  membranes  of  the 
cold  is  distinguished  from  meningitis  by  the  suddenness 


MENINGEAL   HAEMORRHAGE  265 

with  which  the  symptoms  originate  and  by  the  absence 
of  the  more  general  symptoms  that  usually  accompany 
acute  inflammations  of  the  cord.  Otherwise  they 
closely  resemble  each  other. 

The  presence  of  an  injury  or  some  other  distinct 
cause  makes  the  diagnosis  easier.  It  may  be  difficult 
sometimes  to  distinguish  meningeal  haemorrhage  from 
haemorrhage  into  the  substance  of  the  cord,  especially 
as  both  may  be  present  at  the  same  time.  In  the  latter 
there  is  a  tendency  for  the  blood  to  be  extravasated 
into  the  grey  substance,  and  so  to  destroy  sensibility 
to  pain  and  temperature,  leaving  that  to  touch  un- 
impaired. The  diagnosis  may  also  be  assisted  by  the 
appearance  of  the  fluid,  as  shown  by  lumbar  puncture. 

Treatment. — If  there  are  reasons  to  believe  that 
the  blood  is  compressing  the  cord,  the  tension  should  be 
relieved  by  performing  laminectomy.  In  other  cases 
absolute  rest  should  be  enjoined,  and  an  ice-bag  should 
be  applied  to  the  spine.  Calcium  chloride  may  bo  given 
to  promote  clotting. 


SECTION    V.-SYPHILIS    OF   THE 
NERVOUS    SYSTEM 

CHAPTER  XXXI 

INTERSTITIAL    AND    PARENCHYMATOUS 
SYPHILIS 

Syphilitic  disease  of  the  nervous  system  may  be  divided 
into  two  groups  according  as  the  spirochsete  attacks 
chiefly  (a)  the  connective  tissues,  vessels,  and  meninges, 
or  (h)  the  nerve  substance  itself. 

The  lesions  of  the  first  group  are  known  as  inter- 
stitial syphilis,  while  those  of  the  second  are  designated 
parenchymatous  and  in  most  instances  correspond  to  the 
lesions  formerly  described  under  the  title  of  parasyphilis. 
I  The  term  parasyphilis  was  first  used  by  Fournier, 
to  indicate  those  late  results  of  syphilis  which  were 
thought  not  to  be  direct  manifestations  of  the  disease 
in  the  sense  that  secondary  and  tertiary  lesions  were 
considered  to  be,  but  indirect  results  dependent  in  some 
way  on  the  loss  of  vitality  resulting  from  the  presence 
of  toxins. 

The  two  parenchymatous  forms  to  which  most 
attention  has  been  given  are  tabes  and  general  para- 
lysis of  the  insane,  though,  as  will  be  mentioned  later, 
modern  investigation  has  shown  that  Fournier  was 
correct  in  including  several  other  nervous  diseases  in 
this  category.  Evidence  of  the  syphilitic  nature  of 
tabes  and  general  paralysis  has  gradually  accumulated, 
and  in  later  years  has  been  strengthened  in  this  country 
by  the  pathological  work  of  Mott  and  his  insistence  on 
the  fact  that  the  two  diseases  are  identical  in  their 
pathology.     The    discovery    of    the    Wassermann    test 

2GG 


"  PARASYPHILIS  "  267 

forged  a  further  link  in  the  chain  of  evidence,  and  any 
remaining  doubt  was  finally  dispelled  by  Noguchi's 
discovery  of  the  spirochsete  in  the  tissues  of  the  brain 
and  cord. 

Thus  the  diseases  formerly  classed  as  parasyj^hilis, 
and  thought  to  be  indirectly  due  to  syj^hilis,  are  now 
known  to  be  directly  due  to  the  activity  of  the  specific 
organism,  the  Sjnrochceta  pallida  {Sjnronevia  pallidimi) 
and  are  to  be  regarded  as  manifestations  of  the  activity 
of  this  organism.  In  these  circumstances  the  term 
parasyj)hilis  is  no  longer  required,  and  while  it  is  not 
2)ossible  to  make  any  sharp  division  between  lesions  of 
the  connective  tissue  and  those  of  the  nerve  elements, 
owing  to  extension  of  disease  from  one  to  the  other,  it 
has  nevertheless  for  general  clinical  purposes  become 
customary  to  divide  syphilis  of  the  nervous  system  into 
the  two  categories,  viz.  parenchymatous  and  intersti- 
tial, according  to  whether  the  nerve  elements  or  the 
supporting  connective  tissues  are  predominantly  the 
seat  of  the  disease. 

The  diagnosis  of  both  interstitial  and  parenchy- 
matous syphilitic  afiections  of  the  nervous  system  is 
greatly  assisted  by  microscopical  examination  of  the 
cerebro-spinal  fluid  and  by  the  Wassermann  reaction. 

The  cerebro-spinal  fluid. — In  the  cerebro-spinal 
fluid  of  a  normal  person  there  are  scarcely  any  lympho- 
cytes to  be  seen.  In  syphilis  of  the  meninges,  brain,  and 
cord,  lymphocytes  are  frequently  found  in  large  numbers, 
which  vary  from  time  to  time  according  to  the  activity 
of  the  disease,  so  that  from  the  variation  of  their  numbers 
it  is  sometimes  possible  to  gain  some  idea  of  the  effects 
of  treatment ;  but,  as  lymphocytosis  occurs  in  other 
conditions  besides  syphilis,  it  must  not  by  itself  be 
taken  to  be  diagnostic  of  this  disease. 

Changes  in  the  chemical  qualities  of  the  cerebro- 
spinal fluid,  e.g.  an  increase  of  proteid  substance,  have 
been  described  as  a  result  of  syphilis  ;  but  this  cannot 
be  regarded  as  pathognomonic  of  syphilis,  for  it  has  been 
found  also  during  certain  stages  of  dementia  prsecox. 


268    DISEASES   OP  THE   NERVOUS   SYSTEM 

The  Wasscrniauii  test. — A  positive  reaction  of 
the  serum  or  of  the  cerebro-spinal  fluid  to  the  Wasser- 
mann  test  indicates  that  certain  chemical  changes  in  the 
one  or  the  other  have  been  produced  by  the  action  of 
the  spirochaetes  on  the  tissues  of  the  body,  and  it  is 
now  generally  accepted  that,  where  the  tropical  and 
other  diseases  in  which  the  reaction  is  positive  can  be 
ruled  out,  a  positive  reaction  in  either  of  these  fluids  is 
a  definite  indication  of  syphilitic  infection. 

In  locomotor  ataxy  the  reaction  both  in  the  serum 
and  in  the  cerebro-spinal  fluid  obtains  with  considerably 
less  frequency  than  in  general  paralysis,  owing  prob- 
ably to  the  more  chronic  character  of  the  disease  and 
its  greater  liability  to  periods  of  quiescence. 

While  the  Wassermann  reaction  can  usually  be 
modified  rapidly  by  treatment  in  interstitial  syphilis  of 
the  nervous  system,  it  is  only  with  great  difficulty  or 
not  at  all  that  it  can  be  altered  in  the  parenchymatous 
forms,  on  account  apparently  of  the  resistance  which 
the  nerve  tissues  offer  to  the  penetration  of  drugs  that 
affect  the  spirochaetes. 

Mcintosh,  Fildes,  Head  and  Fearnsides  *  have  shown 
that  the  importance  of  the  Wassermann  spinal-fluid  re- 
action does  not  necessarily  end  in  simply  establishing  the 
diagnosis  of  syphilis,  for  the  behaviour  of  the  reaction  can 
be  further  used  to  distinguish  certain  types  of  syphilis 
and  arrive  at  their  prognosis.  For  example,  in  meningo- 
vascular disease  of  the  brain  and  spinal  cord,  these 
authorities  consider  that  the  key  to  the  reaction  of  the 
cerebro-spinal  fluid  lies  in  the  presence  or  absence  of 
inflammatory  changes  in  the  meninges  of  the  spinal  cord 
and  brain  stem,  and  that  disease  of  the  brain  alone 
above  this  level  will  not  give  the  reaction. 

This  point,  if  established,  forms,  as  the  authors 
insist,  an  important  possible  distinguishing  feature 
between  cerebral  meningo- vascular  syphilis  and  general 
paralysis,  for  while  the  cerebro-spinal  fluid  of  general 
paralysis   practically  always   gives  a  positive  reaction, 

*  linnn^  xxxvi.  19i;>  ;niJ  xxxvii.  IDl  \. 


INTERSTITIAL    SYPHILIS    OF    BRAIN     269 

that  of  cerebral  meningo-vascular  syphilis  will  be  negative 
unless  complicated  by  some  lesion  in  the  brain  stem 
and  cord.  Moreover,  when  the  reaction  is  positive  its 
significance  can  be  further  gauged  by  the  efiects  of 
treatment,  for  while  the  reaction  of  meningo-vascular 
syphilis  and  its  clinical  signs  can  usually  be  modified 
by  a  course  of  salvarsan,  those  of  general  paralysis 
rarely  undergo  any  great  change. 

INTERSTITIAL  SYPHILIS 

While  lesions  of  this  form  of  cerebro-spinal  syphilis 
may  appear  at  any  stage,  it  is  important  to  remember 
that  they  may  occur  even  within  a  very  few  months  of 
the  acquisition  of  a  primary  sore,  and  that  they  are  very 
commonly  seen  within  a  period  of  two  years  from  the 
date  of  infection.  According  to  Naunyn,  48  per  cent,  of 
the  cases  occur  within  the  first  three  years.  That  the 
nervous  system  is  often  affected  in  the  very  early  periods 
of  the  disease,  although  there  may  be  no  clinical  signs, 
is  shown  by  the  possibility  of  finding  pleocytosis,  or 
even,  rarely,  a  positive  Wassermann  reaction,  in  the 
cerebro-spinal  fluid  during  the  primary  stages  of  the 
infection.  Men  are  attacked  more  frequently  than 
women,  and  the  cerebral  structures  more  often  than  the 
spinal,  and  when  the  disease  is  apparently  localized  in 
the  cord  there  are  generally  some  signs  to  suggest  that 
the  brain  has  not  altogether  escaped. 

Interstitial  Syphilis  of  the  Brain 

The  structures  chiefly  affected  are  the  vessels,  the 
membranes,  and  the  neuroglia.  It  is  usual  to  find 
the  membranes  and  vessels  affected  at  the  same  time, 
forming  the  condition  termed  syphilis  ineningo-vascularis 
(Head  and  Fearnsides).  Endarteritis  is  often  followed 
by  thrombosis  and  subsequent  softening  of  brain  tissue. 
A  diffuse  gummatous  meningitis,  through  which  the 
brain  is  damaged  by  pressure  or  extension  of  the  inflam- 
mation, is  the  commonest  affection  of  the  membranes. 
Less  commonly,  definite  discrete  gummata  are  found. 


270    DISEASES   OF   THE   NERVOUS   SYSTEM 

Syphilis  meningo-vascularis  very  frequently  begins  at 
the  base  of  the  brain  ;  the  cranial  nerves  are  soon 
damaged,  and  a  special  feature  of  the  disease  is  the 
random  way  in  which  they  are  picked  out.  The  nerves 
supplying  the  ocular  muscles  are  particularly  liable  to 
suffer,  and  ocular  palsies,  often  of  transient  character, 
are  among  the  most  common  symptoms  of  basal  syphilitic 
meningitis.  Pupillary  changes,  apart  from  tabes  and 
general  paralysis — e.g.  inequality  in  size  and  shape  and 
deficiency  in  reaction  to  light — are  common.  Optic 
neuritis  is  frequently  present,  and  when  the  disease  is 
situated  in  the  middle  fossa  there  may  be  other  disturb- 
ances of  vision  due  to  j^ressure  on  the  optic  chiasma.  In 
disease  of  the  posterior  fossa  the  spinal  accessory  and 
hypoglossal  nerves  are  likely  to  be  affected. 

A  gum.ma  may,  of  course,  give  rise  to  the  general  and 
local  signs  common  to  other  forms  of  cerebral  tumour. 

Variation  of  the  symptoms,  intense  headache  which 
is  usually  worse  at  night,  vomiting  and  early  implica- 
tion of  the  cranial  nerves,  are  among  the  signs  that 
suggest  syphilitic  meningitis.  Among  other  symptoms 
of  which  the  patient  may  early  complain.  Head  and 
Fearnsides  mention  shivering  attacks,  with  or  without 
fever,  neuralgic  pains  of  radicular  origin,  and  general 
changes  of  character,  e.g.  want  of  concentration  and  loss 
of  memory. 

Where  the  function  of  the  frontal  lobes  is  modified, 
the  symptoms  may  closely  simulate  those  of  general 
paralysis  of  the  insane,  and  the  resemblance  may  be 
enhanced  by  the  addition  of  convulsions,  more  especially 
when  the  infiltration  of  the  membranes  extends  over 
the  motor  areas.  Such  cases  occurring  in  the  secondary 
period  are  probably  due  to  a  spread  of  the  disease  to  the 
parenchymatous  structures,  and  their  differentiation 
from  the  later  parenchymatous  forms  is  an  important 
matter.  Irregular  patches  of  infiltration,  accompanied 
by  symptoms  which  frequently  disappear,  may  suggest 
the  presence  of  disseminated  sclerosis,  the  more  so  if  at 
the  same  time  patches  of  a  similar  kind  are  disturbing 


INTERSTITIAL    SYPHILIS    OF    CORD      271 

the  functions  of  the  spinal  cord.  The  clinical  picture 
of  cerebral  syphilis  may  show  considerable  changes 
from  time  to  time,  owing  to  the  variation  in  vascularity 
and  intensity  of  inflammatory  processes  in  the  diseased 
parts.  Its  resemblance  to  other  disorders  may  thus 
be  greater  or  less  according  to  the  stage  which  the 
disease  has  reached  at  any  particular  time  ;  and  in 
the  early  stages,  when  there  are  no  definite  signs,  care 
must  be  taken  to  differentiate  it  from  simple  neuras- 
thenia, many  of  the  manifestations  of  which  it  may 
closely  simulate. 

Interstitial  Syphilis  of  the  Spinal  Cord 
As  in  the  case  of  the  brain,  the  structures  chiefly 
affected  are  the  neuroglia,  the  meninges,  and  the  blood- 
vessels.    (Fig.  70.) 


Fig.  70.— Endarteritis  and  periarteritis  of  a  meningeal  artery  from 
a  case  of  syphilitic  meningomyelitis.     {R.  T.  Williamson.) 

The  commonest  lesion  is  that  known  as  meningo- 
myelitis, in  which  there  is  a  diffuse  inflammation  of 
the  neuroglia,  vessels,  and  membranes. 

The  meninges  become  diffusely  infiltrated  with  a 
gummatous  substance  over  an  area  of  var}dng  extent, 
and  at  the  same  time  the  walls  of  many  of  the  arteries 
which  pass  into  the  cord  are  thickened  by  an  inflam- 
matory process  of  a  similar  nature,  which  narrows  or 
altogether  blocks  their  lumen.  The  meningeal  thicken- 
ing causes  symptoms  by  compression  of  the  nerve  roots 
and  other  structures  around,  while  the  narrowing  of  the 
bleed- vessels  impairs  the  circulation  in  the  cord.     The 


272    DISEASES   OF   THE  NERVOUS   SYSTEM 

posterior  arteries  of  the  cord  (Figs.  71,  72)  are  generally 
most  affected,  and  so  the  effects  of  circulatory  disturb- 
ance are  chiefly  visited  upon  the  posterior  columns 
and  the  pyramidal  tracts,  with  the  result  that  the  main 
symptoms  are  those  of  paraplegia,  together  with  some 
degree  of  loss  of  sensation,  to  which  diminished  control 
over  the  bladder  is  often  added. 

Thus,  clinically,  this  condition  shows  itself  in  the 
form  of  an  incomplete  transverse  myelitis  in  which  the 
symptoms,  while  similar  to  those  of  other  forms  of 
myelitis,  are  apt  to  vary  in  their  intensity  from  time  to 


Fig.  71— Section  showing  distribution  of  the  anterioi'  and  pos- 
terior arterial  sj'stems  of  the  cord.  The  part  shaded  by  dots 
shows  the  area  supplied  by  posterior  arteries  of  the  cord. 
{R.  T.  WiUiarnson.) 

time  as  the  condition  of  the  vessels  allows  more  or  less 
blood  to  reach  the  nerve  structures. 

Erb  has  described  a  group  of  cases,  of  gradual  onset, 
in  which  the  motor  columns  suffer  out  of  proportion 
to  the  sensory  tracts,  the  control  of  the  sphincters  being 
also  frequently  weakened.  It  seems  probable  that  in 
some  cases  of  this  description  the  lesion  is  due  to  a 
parenchymatous  systemic  degeneration  of  the  lateral 
and  posterior  columns  and  not  to  a  meningomyelitis, 
but  the  distinction  from  a  clinical  point  of  view,  apart 
from   the  results  of  treatment,  may  be  very  difficult. 


ACUTE   SYPHILITIC   MYELITIS  273 

and  indeed  it  is  possible  that  systemic  degeneration 
and  a  diffuse  myelitic  process  may  coexist. 

Acute  syphilitic  myelitis. — Sometimes  syphilitic 
changes  in  the  cord  come  on  very  rapidly,  and  give 
rise  to  the  clinical  picture  of  acute  transverse  myelitis ; 
indeed,  many  cases  included  under  the  latter  title  are  no 
doubt  instances  of  rapid  softening  of  the  cord  consequent 
upon  syphilitic  endarteritis  and  thrombosis.  The  out- 
look in  such  cases  is  not  good. 

Giuninata. — Isolated  gummata  are  not  common 
either  in  the  membranes  or  in  the  cord  itself.     Their 


i 

Fig.  72.— Diagram  showing  division  of  a  transverse  section  of  the 
cord  into  three  zones  according  to  the  arterial  supply: 
(i.)  Central  zone  (dotted)  in  the  centre  of  the  grey  matter  which 
is  supplied  by  the  anterior  median  or  central  arteries  of  the 
cord  ;  (ii.)  a  peripheral  zone  (shaded)  supplied  by  the  periphernl 
arteries  of  the  cord;  (iii.)  a  third  zone  (pale)  supplied  by  both 
central  and  peripheral  arteries.    {R.  T.  Williamson.) 

symptoms  are  similar  to  those  of  other  tumours,  and 
when  situated  in  the  substance  of  the  cord  they  are  apt 
to  produce  the  Brown-Sequard  form  of  paralysis,  or 
symptoms  which  are  closely  allied  to  it. 

Diagnosis. — The  combination  of  manifestations  that 
are  due  to  disease  of  the  membranes  and  of  the  cord, 
together  with  the  variability  of  the  symptoms,  the 
history  of  a  primary  infection  when  it  can  be  obtained, 
and  the  results  of  the  Wassermann  tests  of  the  blood 
and  spinal  fluid,  are  some  of  the  main  points  to  which 


274    DISEASES   OF   THE   NERVOUS   SYSTEM 

attention  should  be  paid.  A  careful  examination  of  the 
back  should  be  made  in  order  to  exclude  the  presence 
of  caries,  and  the  possibility  of  the  existence  of  a  tumour 
other  than  a  gumma  must  be  remembered. 

Irritation  and  compression  of  the  posterior  roots 
by  the  thickened  meninges  may  cause  symptoms  re- 
sembling those  of  tabes,  but  the  rapidity  with  which 
the  symptoms  arise,  together  with  the  additional  signs 
of  meningitis  and  extension  of  the  disease  to  the  more 
central  structures  of  the  cord,  generally  makes  the  diag- 
nosis clear. 

Prognosis. — When  the  circulation  in  any  part 
of  the  cord  becomes  almost  arrested  there  is  great  danger 
of  softening,  followed  by  a  sclerosis,  which  permanently 
damages  the  cord. 

The  patient  is  also  very  liable  to  relapses. 

The  course  of  the  disease  cannot  be  predicted  until 
the  effects  of  antisyphilitic  treatment  are  seen,  but 
it  may  be  said  generally  that,  when  the  symptoms  are 
of  recent  origin,  the  results  are  fairly  favourable.  In 
some  instances  the  symptoms  disappear  with  great 
rapidity ;  in  others  there  is  permanent  disablement 
from  the  effects  of  secondary  degenerations  ;  in  others 
again  the  paraplegia  becomes  complete  and  complicated 
by  bedsores  and  cystitis,  with  subsequent  death  from 
exhaustion. 

PARENCHYMATOUS  SYPHILIS 

Parenchymatous  syphilis,  as  we  have  seen,  is  the 
term  denoting  the  results  of  infection  of  the  nervous 
elements  of  th#  brain  and  cord  by  the  spirochsetes.  The 
condition  is  mainly  characterized  by  degeneration  of 
tracts  of  neurones,  and  clinically  therefore  the  symp- 
toms differ  in  different  cases  according  to  the  group- 
ing and  the  order  in  which  the  degeneration  takes 
place. 

The  combination  of  symptoms  most  widely  recognized 
is  that  of  tabes,  general  paralysis,  and  the  mixed  cases 
of  tabo-paresis,  but  there  is  no  reason  why  other  com- 


PARENCHYMATOUS    SYPHILIS  275 

binations  should  not  take  place,  and  Head,  Fearnsides,* 
and  others  have  pointed  out  that  some  clinical 
forms  of  progressive  muscular  atrophy,  lateral  and 
combined  sclerosis,  primary  optic  atrophy  and  periodic 
epileptiform  attacks  may  equally  well  be  manifestations 
of  the  disease. 

An  important  fact,  concerning  which  probably  all 
observers  are  agreed,  is  that  in  parenchymatous 
syphilitic  affections  of  the  nervous  system  the  symp- 
toms of  the  original  infection  have  often  been  slight, 
and  even  so  insignificant  as  to  escape  the  atten- 
tion of  the  patient.  The  difficulty  which  frequently 
occurs  in  obtaining  any  history  of  infection  is  well 
known,  and  Mott,|  in  600  post-mortem  examinations 
of  cases  of  general  paralysis,  observed  that  external 
marks  of  syphilis  in  the  shape  of  lesions  of  the  skin, 
bones,  and  viscera  were  relatively  few  in  number.  The 
presence  of  gummata  or  scars  of  gummata  in  the  viscera 
is  also  rare,  and  in  a  considerable  number  of  cases  the 
only  internal  evidence  that  can  be  found  of  previous 
syphilis  is  nodular  fibrosis  of  the  aorta.  It  has  been 
suggested  that  these  cases  are  associated  with  a  type 
of  spirochsete  which  shows  a  peculiar  tendency  to  attack 
the  nervous  system  to  the  exclusion  of  the  other  tissues 
of  the  body,  a  view  which  recent  experimental  work 
eeems  to  support. 

The  difficulty  which  often  exists  in  obtaining  evidence 
of  syphilitic  infection  in  tabes  and  general  paralysis  is 
now  largely  counteracted  by  means  of  the  Wassermann 
tests  of  the  blood  and  spinal  fluid,  which  give  a  positive 
result  in  almost  every  instance  of  general  paralysis  and 
in  a  large  percentage  of  the  cases  of  tabes. 

The  first  stage  of  the  syphilitic  infection  having 
passed,  there  generally  ensues  a  quiescent  period  of 
considerable  length  (an  average  of  about  twelve  years 
in  the  case  of    tabes  and  general  paralysis),  in  which 

*  "  The  Clinical  Aspects  of  Syphilis  of  the  Nervous  System  in  the 
Light  of  the  Wassermann  Reaction  and  Treatment  with  Neosalvarsan," 
by  Henry  Head  and  E.  G.  Fearnsides  ;  Brain,  1914,  vol.  xxxvi.,  Pt.  i. 

■\  Archives  of  Neurology  and  Fsychiatry,  vol.  vi. 


276    DISEASES   OF   THE   NEEVOUS   SYSTEM 

the  spirochaetes  remain  latent,  and  it  has  been  sug- 
gested that  they  may  during  this  period  possibly  assume 
a  granular  form. 

When  activity  recommences,  degenerations  begin  to 
make  their  appearance,  and  the  manifestations  of  paren- 
chymatous syphilis  arise. 

As  to  the  nature  of  the  reactions  which  precede 
the  degenerations,  somewhat  different  views  are  taken. 
That  most  commonly  accepted,  due  largely  to  the  work 
of  Mcintosh  and  Fildes,*  treats  "  parasyphilis  "  as  a 
purely  syphilitic  process  comparable  to  other  forms  of 
tertiary  syphilis  elsewhere. 

We  now  know  that  the  central  nervous  system  is  very 
commonly  infected,  even  in  the  absence  of  symptoms,  in 
the  earliest  stages  of  the  disease  (11  per  cent,  in  late 
primary  syphilis). f  Those  tissues  which  are  affected 
become  "  hypersensitive  "  to  the  virus,  in  the  sense  that 
they  will  respond  to  a  further  intoxication  with  an  abnor- 
mally great  reaction.  Such  increased  susceptibility  of 
the  tissues  is  familiar  in  the  ordinary  gumma  and  in  the 
lesions  of  tuberculosis.  The  spirochaetes  which  caused 
the  sensitizing  infection  in  the  early  stages  apparently 
die  out  after  the  secondary  period  has  passed,  except 
in  certain  areas  w^here  they  persist  as  "  rests."  Such 
areas  are  in  particular  the  wall  of  the  aorta,  the  bones, 
and  the  central  nervous  system.  If  now  the  local  balance 
between  virus  and  immunity  is  upset,  the  spirochsetes  in 
a  rest  will  multiply  and  give  rise  to  a  widesj)read  reaction 
in  the  hypersensitive  tissues.  Should  such  a  reaction 
occur  around  rests  in  the  blood-vessels  of  the  central 
nervous  system,  an  interstitial  lesion  will  result ;  but 
if  the  rest  happens  to  lie  among  the  nerve  tissues, 
the  lesion  will  also  be  parenchymatous.  The  parenchy- 
matous cells  will  react  to  the  intoxication,  but,  having 

*  "A  Comparison  of  the  Lesions  of  Syphilis  ai)d  Parasyphih's. 
together  with  evidence  in  fav(  ur  of  the  identity  of  these  two  condi- 
tions." Brain,  l'.>14,  xxxvii.  141. 

t  P.  Fddes,  R.  J.  S.  Parnell,  and  H.  B.  Maitland.  '  Unsu=!pected 
Involvement  of  the  Central  Nervous  System  in  Syi)hi]i.-,"  Med.  Res. 
Council,  Sjjrc.  liipt.  Scries,  No.  15.   1920. 


PARENCHYMATOUS   SYPHILIS  277 

no  aptitude  for  repair,  will  succumb  and  degenerate. 
The  lost  function  will  then  account  for  the  symptoms  of 
the  disease.  Such  an  explanation  of  the  origin  of  para- 
syphilis  is  closely  comparable  with  the  results  of  a 
parenchymatous  lesion  of  the  testis — irreparable  loss  of 
function. 

The  peculiar  distribution  of  the  lesions  of  parenchy- 
matous syphilis,  namely  the  affections  of  the  posterior 
columns  in  tabes  and  the  j)icking  out  of  particular  areas 
of  the  brain  in  general  paralysis  of  the  insane,  is  probably 
accounted  for  by  a  sj)ecial  distribution  of  the  spirochsetes 
in  the  acute  stage  by  the  j)erineural  lymphatics  con- 
necting the  superficies  of  the  body  with  the  central 
nervous  system.  The  distribution  of  the  syphilitic 
lesions  would  thus  follow  the  same  course  as  the  cord 
lesions  found  in  cases  of  bedsore  by  Orr  and  Rows. 
A  perineural  inflammation  spreading  up  to  the  cord  is  a 
well-known  phenomenon  in  expierimental  rabbit  syphilis 
in  the  early  stages. 

Thus,  according  to  this  hypothesis,  the  stages  which 
lead  up  to  parenchymatous  syphilis  may  be  summed  up* 
as  follows  :■ — (I)  Primary  infection,  (2)  sensitization  of 
the  nerve  tissues,  (3)  recrudescence  of  spirochaetal 
activity  in  hypersensitive  tissues,  (4)  abnormally  active 
response  of  the  hypersensitive  tissues,  (5)  degeneration 
of  neurones. 


CHAPTER  XXXTI 

GENERAL   PARALYSIS   OF   THE    INSANE 
(DEMENTIA    PARALYTICA) 

Etiology. — Men  are  more  liable  to  suffer  from  the 
disease  than  are  women,  and  it  most  commonly  begins 
between  the  ages  of  35  and  55.  A  history  of  previous 
syphilis  can  be  obtained  in  a  large  number  of  cases,  and 
the  results  of  the  Wassermann  test,  together  with  the 
discovery  of  the  presence  of  the  spirochsete  in  the  brain 
tissue  (Plate  14),  have  thrown  a  flood  of  light  on  the 
causation  of  this  disease. 

Wassermann  and  Plant  obtained  a  positive  reaction 
of  the  Wassermann  test  with  serum  in  99*5  per  cent., 
and  with  spinal  fluid  in  95  per  cent,  of  the  cases,  while 
Browning  found  it  in  96  per  cent. 

The  presence  of  the  spirochsete  in  the  brain  of  general 
paralytics  was  first  demonstrated  by  Noguchi,  whose 
observations  were  soon  confirmed  by  Marie,  Levaditi, 
Mott  and  others.  The  organisms  are  found  in  small 
foci,  and  are  generally  most  plentiful  in  the  frontal  lobes. 
While  general  paralysis  must  be  regarded  as  due  to 
syphilis  in  the  vast  majority  of  cases,  if  not  indeed  in 
all,  it  is  generally  accepted  that  in  certain  circumstances 
other  conditions,  such  as  worry,  alcoholism,  and  injury, 
may  precipitate  the  onset  or  accelerate  the  course  of 
the  disease  ;  and  it  has  been  aptly  said  that  general 
paralysis  is  a  disease  of  syphilization  and  civilization. 
Where  any  question  of  this  kind  assumes  a  medico- 
legal aspect,  the  history  and  course  of  the  disease  in 
the  particular  instance  must  be  carefully  considered  and 
the  case  judged  on  its  own  merits. 

278 


.  g  .■ggjtfMBmi 


PLATE  li.— Spirochsetes  in  the  brain,  near  small  vessel, 

in  a  case  of  general  paralysis  of  the  insane,    x  1450. 

{Sir  F.  W.  Mott.) 


GENERAL    PARALYSIS    OF    THE    INSANE    279 

In  some  cases  the  onset  occurs  within  a  few  years 
of  infection,  in  others  it  may  be  delayed  as  long  as 
twenty  or  twenty-five  years,  but  the  average  interval 
between  the  time  of  infection  and  the  onset  of  symptoms 
is  about  ten  or  twelve  years.  As  already  stated,  the 
interstitial  syphihtic  lesions  of  the  brain  often  arise 
within  the  first  two  years  after  infection — a  fact 
which,  in  the  opinion  of  Mott,  suggests  that  the  specific 
organism  of  general  paralysis  may  have  become  in 
some  way  modified.  The  views  concerning  the  paths  by 
which  the  infection  reaches  the  nervous  system  and  the 
course  which  the  degenerative  processes  take  have  been 
discussed  in  the  previous  chapter. 

Juvenile  general  paralysis. — In  a  small  percentage 
of  the  cases  the  disease  arises  in  young  people  at  about 
or  soon  after  the  age  of  puberty,  and  of  these  congenital 
syphilis  is  considered  to  be  the  cause. 

Pathology. — To  the  naked  eye,  atrophy  of  the 
cerebral  convolutions,  especially  those  of  the  frontal 
lobes,  and  thickened  and  adherent  membranes,  are 
among  the  principal  changes.  The  tendency  for  the 
frontal  lobes  to  sufier  out  of  proportion  to  the  rest  of  the 
brain  is  interesting,  and  according  to  Mott  it  may  perhaps 
be  accounted  for  by  the  anatomical  arrangement  of  the 
veins,  which  tends  to  produce  a  venous  congestion  of  the 
circulation  in  this  area.  With  the  microscope,  degenera- 
tion of  cells  and  tracts  of  fibres  connecting  various  parts 
of  the  cortex  with  one  another  (association  fibres)  can 
be  demonstrated.  The  cortical  cells,  especially  the  Betz 
cells  of  the  motor  area,  are  swollen  and  altered  in  shape, 
and  in  the  later  stages  of  the  disease  many  of  them 
disappear,  their  place  being  taken  by  an  overgrowth  of 
neurogha. 

The  vascularity  of  the  cortex  is  increased,  and  new 
capillary  formation  can  be  seen.  The  endothelial  cells 
of  the  capillary  walls  proHferate,  and  the  perivascular 
spaces  are  filled  with  lymphocytes  and  plasma  cells. 
The  origin  of  the  plasma  cells  is  doubtful,  but  their 
presence   is    very   characteristic    of    general    paralysis. 


280    DISEASES   OF   THE  NERVOUS   SYSTEM 

In  addition  there  are   "  mast  "   cells  and  "  rod "  cells 
to  be  seen. 

Symptoms. — The  first  symptoms  are  usually  those 
connected  with  the  mind.  The  patient  becomes  irrit- 
able, unstable,  and  alternately  unduly  depressed  and 
exalted.  Early  mental  changes  vary  in  character  in 
different  individuals  :  in  some  patients  depression  is  a 
feature ;  in  others  exaltation  is  present.  Convulsive 
seizures,  though  commoner  in  the  later  stages,  are  some- 
times among  the  first  symptoms  to  be  noticed. 

Forgetfulness  is  especially  apt  to  be  noticed  as  an 
early  symptom  when  the  patient  is  a  business  man,  for 
naturally  any  mistakes  are  soon  liable  to  be  found  out  ; 
but  where  the  patient  has  not  a  responsible  position,  loss 
of  memory,  although  perhaps  present,  is  more  likely  to 
be  overlooked.  The  same  is  true  of  manual  work  ;  the 
last  and  most  delicate  acquirements  are  the  first  to  decline, 
on  account  of  failure  of  the  attention  and  of  the  delicate 
coordination  that  arc  required  for  their  performance. 

When  the  patient  is  brought  for  examination  there 
are  frequently  definite  signs  which,  taken  with  the 
mental  condition,  make  the  nature  of  the  case  clear. 
Of  these  the  most  common  are  inequality  of  the  pwpils 
with  failure  to  react  to  light,  a  "  slurring  "  speech,  and 
tremors  of  the  lips,  tongue,  and  hands. 

The  pupils  are  like  those  of  locomotor  ataxy  (Argyll- 
Robertson),  and  contract  during  accommodation  but 
not  to  light.  Their  inequality  is  often  marked,  and  they 
have  often  ceased  to  be  circular,  and  exhibit  an  "  angu- 
lar '*  appearance.  There  is  no  nystagmus,  and  in  the. 
early  stages  the  optic  discs  do  not  usually  show  any 
change,  although  later  on  they  may  undergo  atrophy. 

The  speech  is  generally  tested  by  the  ability  to  say 
such  words  as  "  artillery,"  "  constitutional,"  and  "  con- 
stabulary." There  is  a  great  tendency  to  elide  syllables, 
a  tendency  which  is  also  noticed  in  the  writing  (Fig.  73). 
In  talking  there  is  a  fine  tremor  of  the  lips  and  a  general 
over-action  of  all  the  face  muscles  ;  a  tine  tremor  may 
also  be  observed  in  the  tongue. 


GENERAL   PARALYSIS   OF   THE    INSANE    281 

The  hands  tremble,  and  there  is  loss  of  accuracy  in 
movements  requiring  fine  coordination,  as  can  often  be 
demonstrated  by  asking  the  patient  to  button  up  his  coat. 

The  tremor,  incoordination,  and  general  failure  of 
intellect  are  often  all  made  manifest  in  the  writing 
(Fig.  73). 

The  knee-jerks  may  be  absent  or  increased,  and  in 
the  latter  event  they  are  often  unequal;  the  plantars 
may  give  an  extensor  response. 

Diagnosis. — General  paralysis  of  the  insane  is  often 
seen  by  the  neurologist  during  its  early  stages,  when 
it  is  most  important  to  arrive,  if  possible,  at  an  accu- 
rate diagnosis.  In  the  early  stages,  care  must  be  taken 
to  avoid  confusion  with  neurasthenia.  Occasionally  it 
may  at  first  be  impossible  to  make  a  certain  diagnosis, 


Fig.  73.— Specimen  of  writing  from  a  case  of  general  paralysis. 

but  usually  there  is  a  difference  between  the 'simple 
fatigue  symptoms  of  neurasthenia  and  the  mental  de- 
generation of  general  paralysis  ;  and  with  the  develop- 
ment of  physical  signs  the  diagnosis  becomes  clear. 
The  neurasthenic  worries  about  himself,  but  in  general 
paralysis  it  is  the  friends  who  are  often  first  worried  on 
account  of  the  mental  symptoms  which  change  the  man 
from  his  former  self  into  an  irritable,  careless,  and 
strange  being. 

Interstitial  forms  of  cerebral  syphilis  are  often  very 
difficult  to  distinguish  from  general  paralysis  ;  and  the 
methods  which  may  assist  in  the  differentiation  have 
already  been  discussed  (p.  268).  That  the  two  should 
be  distinguished  is  most  important,  for  while  the  prog- 
nosis of  the  meningo-vascular  form,  if  it  be  adequately 
treated,  is  comparatively  good,  that  of  general  paralysis 
is  almost  hopeless. 


282    DISEASES   OF   THE   NERVOUS   SYSTEM 

This  disease  may  also  bear  seme  resemblance  in  its 
early  stages  to  disseminated  sclerosis,  the  chief  symptoms 
common  to  both  being  tremors,  alteration  in  speech, 
and  increase  of  deep  reflexes  ;  but  it  must  be  remem- 
bered that  the  knee-jerks  are  by  no  means  always 
increased  in  general  paralysis — ^they  may  be  diminished, 
unequal,  or  absent.  The  tremor  of  general  paralysis 
is  finer  than  that  of  disseminated  sclerosis,  and  it  is, 
moreover,  especially  marked  in  the  face,  tongue,  and 
lips.  The  speech,  too,  when  well  developed,  differs  in  the 
two  conditions  :  in  general  paralysis  it  is  hesitating  and 
syllables  are  dropped  or  telescoped  into  one  another ; 
while  in  disseminated  sclerosis  it  is  jerky,  staccato,  or 
scanning — characters  which  are  rather  difficult  to  de- 
scribe, but  which  are  distinctive  when  heard. 

Among  other  conditions  which  may  sometimes  closely 
simulate  general  paralysis  are  some  phases  of  mental 
disorder  due  to  chronic  alcoholism.  Tumours  of  the 
frontal  lobes  with  mental  symptoms  and  tremors  may 
also  bear  some  resemblance  to  it,  but  the  presence  of 
optic  neuritis  and  the  absence  of  Argyll-Robertson 
pupils  help  to  serve  as  distinguishing  features. 

Prognosis. — The  prognosis  is  always  bad,  and  the 
patients,  w^ith  the  exception  of  occasional  remissions, 
tend  to  go  steadily  downhill.  Fits,  epileptiform  or 
apoplectiform,  are  often  a  marked  feature,  and  each 
attack  generally  leaves  the  patient  both  physically 
and  mentally  worse  than  he  was  before.  As  the  mind 
deteriorates  the  physical  weakness  increases,  until  at 
last  the  patient  arrives  at  the  state  which  justifies  the 
use  of  the  term  "  general  paralysis." 

Treatment.  —  That  the  treatment  of  general 
paralysis  is  not  usually  followed  by  satisfactory 
results  is  due  chiefly,  it  would  appear,  to  the  difficulty 
which  the  drugs  encounter  in  reaching  the  spirochaetes. 

It  has  long  been  known  that  no  good  results  can  be 
expected  from  giving  mercury,  and  at  present  but  little, 
if  any,  more  benefit  can  be  obtained  from  salvarsan. 
Various    methods    of    bringing    the    latter    drug    into 


GENEEAL    PARALYSIS    OF   THE    INSANE    283 

closer  contact  with  the  spirochsetes  have  been  tried,  the 
chief  of  which  is  that  proposed  by  Swift  of  injecting 
immunized  serum  into  the  cerebro-spinal  fluid,  into 
which  salvarsan  itself  cannot,  of  course,  be  introduced. 
Various  modifications  of  this  method  have  been  tried, 
e.g.  subdural  injections  and  injections  into  the  lateral 
ventricles,  but  unfortunately  up  to  the  present  with 
little  definite  success. 

Treatment  otherwise  consists  in  placing  the  patient 
under  proper  care  and  control,  giving  attention  to  the 
general  health,  and  trying  to  relieve  individual  symptoms 
as  they  may  arise. 


CHAPTER   XXXTII 

TABES   DORSALIS  (LOCOMOTOR   ATAXY) 

Etiology. — The  symptoms  of  locomotor  ataxy  are 
due  principally  to  degeneration  of  the  lowest  sensory 
neurones.  But  although  the  degeneration  mainly  attacks 
the  sensory  neurones,  some  signs  of  its  extension  to  those 
of  motor  function  also  are  often  seen,  more  especially  in 
connection  with  the  ocular  nerves,  and  occasionally, 
though  less  frequently,  in  connection  with  motor  nerves 
in  other  parts  of  the  body. 

The  disease  is  found  in  men  about  ten  times  as  often 
as  in  women,  and  the  onset  in  most  cases  occurs  some- 
where between  the  ages  of  20  and  50.  The  time  at  which 
symptoms  appear  after  the  primary  infection  is  very 
variable  and  may  be  as  long  as  twenty-five  years  or 
more,  but  the  average  is  about  ten  or  twelve  years. 
Occasionally  patients  with  the  disease  are  seen  while  in 
their  teens,  and  to  these  cases  the  term  juvenile  tabes  is 
applied.  Though  it  is  possible  that  injury,  alcoholism, 
worry  and  other  debilitating  influences  may  act  as 
contributory  causes,  the  application  of  the  Wasser- 
mann  test  and  the  discovery  of  spirochsetes  in  the  central 
nervous  system  have  placed  the  dependence  of  loco- 
motor ataxy  upon  a  previous  attack  of  syphilis  beyond 
all  reasonable  doubt,  and  have,  moreover,  demonstrated 
a  direct  relationship  between  the  two  conditions  ;  for 
while  formerly  it  was  thought  that  syphilis  acted  in- 
directly by  lowering  the  vitality  of  the  neurones,  it 
is  now  known  that  the  action  is  directly  due  to  the 
activity  of  the  spirochaetes,  and  that  tabes  dorsalis  is  to 

281 


TABES   DOESALIS:    ETIOLOGY  285 

be  regarded  as  one  of  the  parenchymatous  forms  of 
syphilis  of  the  nervous  system. 

The  reason  for  the  definitely  selective  action  of  the 
toxin  on  the  lowest  sensory  neurones  has  been  much 
debated.  It  is  well  known  that  poisons  Hke  lead, 
diphtheria,  alcohol,  and  arsenic  all  exercise  a  selective 
action  on  certain  groups  of  fibres,  and  it  may  be  that 
the  poison  which  causes  tabes  acts  in  a  similar  manner. 

One  view  is,  that  the  poison  injures  the  cell  bodies 
from  which  the  fibres  arise  and  receive  their  nutrition. 
In  this  instance  the  cell  bodies  are  situated  in  the 
posterior  root  ganglia,  and  changes  in  them  have  been 
described,  although  they  do  not  appear  to  be  demon- 
strable in  every  case.  Moreover,  if  failure  of  the  nutri- 
tion of  these  cells  causes  degeneration  of  the  central 
process  of  the  T-shaped  fibres  which  spring  from  them, 
one  would  expect  the  peripheral  process  to  degenerate 
as  well,  but  this  does  not  by  any  means  always  occur. 
Changes  have,  however,  been  described  in  the  muscle 
spindles  in  which  the  periphery  of  some  of  the  nerves 
terminates,  and  it  may  be  that  these  are  the  first  indica- 
tion of  a  peripheral  degeneration  (Batten). 

There  are  anatomical  reasons  which  seem  to  make 
the  fibres  of  the  posterior  nerve  roots  comparatively  less 
resistant.  There  is,  for  example,  some  slight  natural  con- 
striction of  the  roots  as  they  pass  through  the  pia  mater. 
This  has  been  thought  to  lower  their  resisting  powers, 
and  some  neurologists  consider  that  the  degeneration 
is  produced  by  a  thickening  of  the  spinal  membranes 
which  compresses  the  roots  as  they  pass  through  the  pia 
mater,  i.e.  at  the  point  at  which  they  are  naturally 
rather  narrowed,  and  that  this  thickening  is  caused  by 
a  tertiary  syphilitic  process.  That  some  thickening 
of  the  membranes  is  frequently  present  is  undisputed, 
but  it  is  difficult  to  conceive  it  as  the  main  cause  of 
the  degeneration.  If  it  were  so  one  would  expect  the 
anterior  roots  to  suffer  more  frequently ;  and,  further, 
there  is  also  the  difiiculty  of  accounting  for  the  ocular 
and  other  cranial  nerve  symptoms  on  this  hypothesis. 


286     DISEASES   OF   THE   NERVOUS   SYSTEM 

The  part  whicli  the  lymphatics  play  in  the  selective 
nature  of  the  degeneration  is  probably  very  important, 
and  the  hypothesis  concerning  the  sensitization  of  the 
posterior  columns  has  already  been  discussed  in  the 
section  dealing  with  the  general  aspect  of  parenchy- 
matous syphihs  (pp.  276,  277). 

Orr  and  Rows  *  have  published  the  results  of  some 
important  researches  on  this  subject,  which  show  that 
"  in  peripheral  nerves,  spinal  roots,  and  cranial  nerves 
there  is  a  constant  stream  of  lymph  ascending  towards 
the  central  nervous  system,  whose  main  current  lies  in 
the  inner  meshes  or  lymph  spaces  of  the  fibrous  perineural 
sheath."  Toxins  reaching  the  spinal  cord  and  brain  by 
this  channel  are  particularly  apt  to  affect  the  nerves 
where  they  have  lost  their  neurilemma  sheath,  which 
spot,  Orr  and  Rows  point  out,  is  not  quite  identical 
with  the  place  at  which  the  nerves  undergo  a  slight 
constriction  as  they  pass  into  the  cord.  In  this  way  the 
distribution  of  the  lymph  may  be  the  determining  cause 
of  the  locality  of  the  degeneration,  and  it  is  possible, 
as  suggested  by  Mott,  that  the  immediate  cause  of  the 
degeneration  is  produced  not  always  by  the  organisms 
themselves,  but  by  a  proteolytic  ferment  which  arises 
outside  the  cord  and  then  passes  up  to  the  cord  by 
the  lymphatics  of  the  jDosterior  roots.  Thus  the  steps 
of  the  process  would  be  :  (1)  focus  of  infection  ;  (2) 
production  of  proteolytic  ferment  ;  (3)  circulation 
of  ferment  in  the  lymphatics  of  the  posterior  roots  ; 
(4)  degeneration  of  the  nerve  fibres. 

Pathology. — It  is  generally  agreed  that  degeneration 
of  the  nerve  fibres  is  the  primary  event,  and  that  the  over- 
growth of  neuroglia  is  secondary,  and  this  view  receives 
confirmation  from  the  fact  that  the  only  degenerated 
fibres  traceable  in  the  cord  during  the  early  stages  of  the 
disease  are  those  which  are  continuous  with  the  roots 
(i.e.  exogenous  fibres),  and  not  those  which  pass  from 
one  part  of  the  cord  to  another  (i.e.  endogenous  fibres). 
Were  the  primary  condition  an  overgrowth  of  neuroglia, 

*  Jonrn.  of  NetiroL  and  rsychiatry,  May,  1907. 


TABES   DOKSALIS:    PATHOLOGY 


287 


no  such  distinction  would  be  shown  between  the  different 
sets  of  fibres. 

Of  these  fibres  from  the  posterior  roots  which  are  so 
prone  to  degenerate,  some  proceed  up  the  posterior 
columns,  some  complete  the  reflex  arcs  by  communi- 
cating with  the  cells  of  the  anterior  horns,  and  some 
terminate  in  Clarke's  column,  from  which  fibres  arise  to 
pass  up  the  direct  cerebellar  tracts  (Fig.  74,  a,  b,  and  c), 
while  others  end  in  the  cells  of  the  grey  matter. 

Degeneration  is  most  prominently  marked  in  con- 
nection with  those  fibres  which 
pass  up  the  posterior  columns 
of  the  cord,  and  if  the  course 
of  a  single  degenerated  fibre 
be  traced  it  will  be  observed 
first  to  pass  up  the  posterior 
external  column  (Burdach), 
subsequently  to  find  its  way 
into  the  posterior  internal 
column  (Goll),  and  then  to 
pass  up  to  the  medulla,  where 
it  terminates. 

Thus  a  section  of  the  cord 
shows  degeneration  of  the  pos- 
terior columns,  and,  since  the 
roots  in  the  lower  part  of  the 
cord  are  generally  the  most 
affected,  the  degeneration  is 
there  most  dense  in  the  posteiicr  external  columns, 
while  it  gradually  becomes  more  pronounced  in  the 
posterior  internal  columns  as  it  ascends  the  cord. 

Mechanism  by  which  symptoms  are  pro- 
duced.— The  symptoms  of  locomotor  ataxy  being  the 
result  of  degeneration  of  fibres  in  the  posterior  nerve 
roots,  consideration  of  the  functions  of  these  fibres  and 
of  the  consequences  following  their  destruction  will  give 
a  good  idea  of  the  different  symptoms  that  occur. 

A  posterior  nerve  root  contains  (1)  fibres  carrying 
impulses  for  sensations  of  touch,  pain,  and  temperature  ; 


Fig.  74 


Diagram  showing 
the  inain  destinations  of 
fibres  of  the  posterior 
nerye  roots.     (See  text.) 


288     DISEASES   OF   THE   NERVOUS   SYSTEM 

(2)  fibres  along  which  impressions  pass  from  the  muscles, 
ligaments,  and  joints  ;  and  (3)  fibres  which  complete 
the  reflex  arcs  by  communicating  with  the  cells  in 
the  anterior  horns. 

(1)  Failure  of  nutrition  of  the  fibres  which  convey 
impressions  of  touch,  pain,  and  temperature  results  in 
anaesthesia  over  the  areas  of  skin  corresponding  to  the 
particular  roots  affected  ;  but  since  all  the  fibres  in  the 
root  are  not  damaged  to  an  equal  extent,  until  the  root 
is  entirely  destroyed  the  anaesthesia  occurs  in  patches, 
some  part  of  a  given  root-area  being  more  anaesthetic 
than  others. 

Further,  one  form  of  sensation  may  suffer  more 
than  another,  so  that  the  patches  of  anaesthesia  to  touch, 
pain,  and  temperature  do  not  necessarily  coincide. 

This  degeneration  is  accompanied  by  signs  of  irrita- 
tion of  the  nerves  which  takes  the  form  of  "  lightning 
pains  "  and  visceral  crises. 

The  loss  of  recognition  of  sensory  impulses  is  closely 
associated  with  modifications  of  nutrition  in  the  skin, 
bones,  and  joints,  constituting  the  so-called  trophic 
changes. 

(2)  The  degeneration  of  fibres  which  should  convey 
impulses  from  the  muscles,  ligaments,  and  joints  is  ac- 
companied by  incoordination  and  a  failure  to  recognize 
the  relative  positions  in  which  various  parts  of  the 
limbs  are  placed  (i.e.  loss  of  sense  of  position).  The 
power  of  distinguishing  the  forms  of  objects  is  also 
frequently  diminished  (astereognosis). 

(3)  Degeneration  of  the  fibres  which  communicate 
with  the  cells  of  the  anterior  horns  leads  to  loss  of  reflexes 
and  to  loss  of  tone  of  the  muscles.  The  impulses  which 
constantly  flow  into  the  cord  through  the  posterior 
roots  exercise  a  definite  influence  upon  those  flowing 
out  by  the  anterior  roots,  and  if  they  are  cut  off  either 
by  experiment  or  by  disease  as  they  are  in  the  case  ol 
locomotor  ataxy,  the  tone  or  elasticity  of  the  muscles 
of  the  corresponding  arc  is  diminished,  and  the  con- 
dition known  as  hypotonus  arises  (Fig.  75). 


TABES   DORSALIS 


289 


If  these  principles  are  applied  to  different  levels 
of  the  cord  the  mechanism  of  the  main  symptoms  is 
clearly  demonstrated. 

Thus  from  degeneration  of  the  lumbar  and  sacral 


Fig.  75.— Case  showing  increased  range  of  movement  due  to 
hypotonus  of  the  muscles. 

roots  there  arise  pains  in  the  legs,  patches  of  anaesthesia, 
numbness  and  a  feeling  as  though  walking  on  cotton- 
wool, trophic  symptoms,  incoordination,  loss  of  the 
knee-jerks  and  tendo-Achillis  jerks,  impairment  of  the 
action  of  the  sphincter  reflexes,  and  hypotonus. 

T 


290    DISEASES   OF   THE    NERVOUS    SYSTEM 

In  the  dorsal  region  the  degenerating  fibres  are  apt 
to  cause  girdle  pains  and  a  zone  of  altered  sensation  in 
the  corresponding  course  of  distribution  of  the  roots. 

Higher  still — in  the  cervical  region — there  are  signs 
in  the  arms  corresponding  in  character  to  those  already 
enumerated  for  the  legs  ;  and  at  a  still  higher  level  the 
reflex  arc  for  light  is  broken  and  the  optic  nerve 
atrophies. 

Such,  then,  is  the  general  mode  of  production  of  the 
sensory  symptoms  of  locomotor  ataxy,  over  and  above 
which  there  may  be  many  others  referable  to  alterations 
in  the  motor  neurones. 

General  clinical  course. — It  will  be  clear  from 
what  has  already  been  said  that  there  can  be  no  constancy 
concerning  the  order  in  which  the  different  symptoms 
make  their  appearance. 

The  onset  is  generally  associated  with  fains,  which, 
as  might  be  expected,  may  be  felt  for  a  long  time,  even 
for  years,  before  the  attention  of  the  patient  is  called 
to  any  other  symptom,  though  it  must  be  admitted  that 
a  systematic  examination  would  probably  often  reveal 
the  existence  of  other  signs  at  an  earlier  date  than  they 
are  otherwise  recognized.  The  pains  are  of  a  lightning 
or  shooting  character,  and  vary  greatly  in  their  intensity 
and  duration,  often  disappearing  for  weeks  together, 
to  return  subsequently  for  several  days  at  a  time.  Asso- 
ciated with  these  there  may  be  hyperaesthesia  of  such 
intensity  that  the  patient  cannot  bear  the  slightest  touch. 
At  first  the  pains  are  almost  invariably  attributed  to 
neuralgia  or  rheumatism,  or,  if  they  remain  for  a  time 
confined  to  the  course  of  a  particular  nerve,  they  are 
liable  to  be  attributed  to  a  localized  neuritis,  e.g.  of  the 
sciatic  or  ulnar  nerve; 

In  addition  to  these  lightning  pains  which  are  felt 
in  the  more  superficial  structures,  there  may  be  deeper 
"  boring  "  pains  in  the  bones  or  other  organs,  which 
often  remain  for  a  long  time  localized  to  one  spot. 
Cramps  in  the  muscles  arc  also  sometimes  a  prominent 
feature. 


TABES   DORSALIS:    CLINICAL    COUKSE    291 

Since  the  lumbar  and  the  sacral  roots  are  generally 
among  the  first  to  degenerate,  the  Jcnee- jerks  and  the 
tendo-Achillis  jerks  are,  as  a  rule,  lost  or  much  diminished 
quite  early  in  the  course  of  the  disease,  and  at  the 
same  time  changes  are  very  frequently  occurring  at 
the  other  end  of  the  system  which  result  in  loss  of  the 
light  reflex. 

Thus  the  combination  of  symptoms  most  commonly 
found  early  in  the  disease  consists  of  lightning  pains, 
loss  of  knee-jerks,  and  the  Argyll-Robertson  pupils,  i.e. 
pupils  which  contract  during  accommodation  but  do  not 
react  to  light,  and  which  may  be  pin-point  or  unequal 
in  size. 

In  addition,  if  carefully  looked  for,  there  is  a  likeli- 
hood of  some  degree  of  ataxy  being  detected  in  the  lower 
extremities  ;  but  other  symptoms  often  exist  for  a  long 
time  before  ataxy  is  noticed,  so  much  so  that  a  "  pre- 
ataxic  stage  "  of  the  disease  is  clinically  recognized. 

Another  early,  and  not  infrequently  the  first,  symp- 
tom is  that  of  'primary  atrophy  of  the  optic  nerves. 

The  clinical  course  of  the  disease  may  best  be  described 
by  following  the  development  of  the  chief  symptoms 
(Fig.  76)  in  a  typical  case. 

If  the  patient  comes  complaining  of  "  rheumatic  " 
pains,  which  on  inquiry  prove  to  be  of  a  lightning 
character,  and  examination  shows  loss  of  knee-jerks 
and  the  presence  of  the  Argyll-Robertson  pupil,  the 
diagnosis  of  tabes  in  the  "  preataxic  "  stage  will  prob- 
ably be  made. 

In  such  a  case  other  signs  will  in  all  probability 
gradually  show  themselves,  although  in  favourable 
circumstances  the  progress  of  the  disease  may  be  very 
slow  or  may  even  become  arrested. 

If  degeneration  progresses,  the  patient's  attention 
will  sooner  or  later  be  attracted  by  his  unsteadiness  both 
when  walking  and  standing,  this  unsteadiness  being 
greater  in  the  dark  or  on  such  occasions  as  he  may  have 
his  eyes  shut,  e.g.  when  washing  his  face.  Before,  how- 
ever, the  patient  recognizes  any  difficulty  in  this  direction 


292    DISEASES   OF   THE   NERVOUS   SYSTEM 

it  can  generally  be  discovered  by  making  him  stand  with 
his  feet  together  and  his  eyes  closed  (Romberg's  sign), 
or   by   asking  him  to  try  to   execute   such  balancing 


Argyll-Robertson 

pupil 
Optic  atrophy 
Ptosis 
Ocular  palsies 


Laryngeal 
s  and 

( gastric  cx'ises 


Zone  of    1 
iinsesthesia/ 


(  Sphincter 
\    trouble 


Charcot's ) 
joint     3 


Ataxy 


(  Loss  of  knee- 
(  jerks 


( Lightning 
^  -  *■(      pains 

-.  ^     (  Patches  of 
*  (anaesthesia 


'  j  Loss  of  tendo- 
"'(   Achillis  jerk 


Perforating  ">. 
ulcer        t 


Fig.  76.- Scheme  of  the  principal  symptoms  of  tabes  dorealis. 


TABES  DOKSALIS:   CLINICAL  COURSE     293 

luovements  as  are  necessary  for  walking  with  tlie  heel 
of  one  foot  placed  at  each  step  in  front  of  the  toes  of 
the  other — a  very  delicate  test  if  performed  with  the 
eyes  closed. 

Various  defects  in  the  sensations  of  touch,  pain, 
and  temperature  will  be  found  in  irregular  patches 
corresponding  to  the  areas  supplied  by  the  degenerating 
nerve  roots,  and  the  sole  of  the  foot  is  a  particularly 
likely  place  to  find  such  patches.  The  areas  of  loss  to 
one  kind  of  sensation  do  not  necessarily  correspond  to 
those  of  another,  as  is  only  to  be  expected  when  it  is 
remembered  that  the  separate  nerve  fibres  are  degenerat- 
ing independently  of  one  another. 

Another  sensory  disturbance  often  found  quite  early 
in  the  disease  is  a  zone  of  analgesia  or  anaesthesia  round 
the  chest  or  abdomen,  indicating  that  degeneration  is 
proceeding  in  some  of  the  dorsal  roots. 

Sexual  power  is  usually  greatly  diminished  or  lost 
by  the  time  the  disease  is  fully  developed,  but  during  the 
early  stages  it  may  be  temporarily  increased. 

If  the  case  be  progressive,  the  ataxy  now  increases  in 
degree,  until  at  last  the  patient  may  be  quite  unable 
to  walk  or  even  to  stand.  Before,  however,  this  stage 
is  reached  many  symptoms  may  or  may  not  develop 
incidentally  in  an  order  which  cannot  possibly  be 
prognosticated,  for,  as  already  insisted  upon,  almost 
any  one  of  all  the  possible  symptoms  occurring  during 
the  course  of  the  disease  may  be  the  first  to  attract 
attention. 

With  this  general  sketch  of  the  disease  it  is  now 
necessary  to  consider  certain  of  the  main  symptoms  in 
some  detail. 

Trophic  disturbances.  Perforating  ulcers. — Perforating 
ulcers  are  characterized  by  the  formation  of  crateriform 
indolent  sores  of  various  depths,  which  make  but  little 
attempt  to  heal.  (Fig.  77.)  The  skin  at  their  edges  is 
usually  thickened  and  raised  to  form  a  dense  callosity, 
which  often  exists  as  a  corn  before  any  ulceration 
takes  place. 


294    DISEASES   OF   THE  NERVOUS   SYSTEM 

The  ulcers,  wliieli  tend  to  assume  a  somewhat  conical 
shape,  are  generally  painless,  and  frequently  extend  right 
down  to  the  bone. 

Their  favourite  position  is  on  the  fore  part  of  the 
sole  of  the  foot,  but  sometimes  they  appear  on  the 
upper  surface  of  the  toes  ;  they  show  no  disposition 
whatever  to  occur  on  the  heel. 


Fig.  77.— Perforating  ulcer  in  a  case  of  tabes  dorfcalis. 


The  underlying  factor  in  the  formation  of  these 
ulcers  is  undoubtedly  an  alteration  in  the  nutrition 
of  the  affected  parts  consequent  upon  the  destruction 
of  sensory  fibres,  while  pressure  and  injury  presumably 
take  a  share  in  the  causation.  In  some  cases,  as  suggested 
by  Tourette,  the  ulceration  may  possibly  be  secondary 
to  diseased  bones  and  joints,  and  may  be  in  fact  a  fistula. 
Under   favourable   conditions,   the   most   important   of 


;    TABES   DORSALIS :    TROPHIC    CHANGES    295 

which  is  rest,  an  ulcer  often  heals  spontaneously,  but 
sometimes  first  requires  such  stimulation  as  is  obtained 
by  scraping  or  by  exposure  to  the  X-rays,  the  latter 
mode  of  treatment  appearing  in  some  cases  to  act  very 
favourably. 

Osteo-arthropatMes. — Changes  in  the  bones   and  the 


Fig.  78.— Charcot's  disease   of   the   right 
knee. 

joints  have  next  to  be  considered — the  so-called  osteo- 
arthropathies. 

The  condition  known  as  Charcots  joint  may  occur 
at  practically  any  period  of  the  disease,  and  is  char- 
acterized by  a  painless  and  often  rapid  swelling  due  to 
the  efiusion  of  fluid,  which,  after  being  absorbed,  often 
leaves  the  joint  in  a  flail-Hke  condition,  due  partly  to 
the   laxity  and  softening  of  the   Hgaments  and  partly 


296    DISEASES   OF   THE   NERVOUS   SYSTEM 

to  changes  in  the  articular  surfaces  of  the  bones  ;  these 
last-mentioned  changes  may  all  occur  without  any 
definite  degree  of  effusion.  The  knee-,  elbow-,  hip-, 
and  shoulder- joint  are  among  those  which  most  com- 
monly suffer,  while  another  common  situation,  perhaps 
not  so  often  recognized,  is  that  of  the  big-toe  joint. 
(Figs.  78,  79.)  _ 

The    essential   process  in  all  these  joints  is  one  of 


Fig. 


TU.-Cliavcot's    disease    of    the   right    ankle-joint    associated 
with  atrophic  changes  in  the  bones  of  the  foot. 


atrophy,  which  is  sometimes  accompanied  by  irregular 
bony  outgrowths  similar  to  those  associated  with  rheu- 
matoid arthritis.  This  latter  form,  to  which  the  term 
hypertrophic  is  sometimes  applied,  appears  to  occur  more 
often  in  the  knee-joint  than  elsewhere,  while  the  shoulder 
and  hip  are  more  likely  to  show  signs  of  advanced  atrophy, 
as  evidenced  by  dislocation  of  the  heads  of  the  bones. 

The  exact  mechanism  by  which  these  joint  troubles  are 
produced  is  obscure.     The  influence  of  traumatism  as 


TABES  DOKSALIS:  TROPHIC  CHANGES  297 

a  direct  cause  can  be  largely  excluded  by  their  frequent 
occurrence  in  the  preataxic  stage  of  the  disease,  and  the 
possibiHty  of  their  being  of  syphilitic  origin  is  negatived 


Fig.  80.— Atrophy  of  the  head, 
of    the   humerus    asso- 
ciated witli  a  "  Charcot's 
joint."   (MidcUesex  Hospital . 
Museum.) 


by  the  fact  that  changes  of  practically  the  same  nature 
are  to  be  found  in  syringomyelia,  concerning  which 
there  appears  to  be  no  question  of  the  influence  of 
syphilis. 


298     DISEASES   OF   THE   NERVOUS   SYSTEM 

The  hones. — In  locomotor  ataxy  the    proportion  of 
the  lime  salts  in  the  bones  is  frequently  reduced,  hence 


6C 


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bo 

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o 

bo 

I 


TABES   DORSALLS:    TROPHIC    CHANGES    299 

the  bones  become  soft  and  brittle,  and  "  spontaneous  " 
fractures  (i.e.  fractures  brought  about  by  a  very  slight 
amount  of  violence)  arise. 

The  atrophy  of  bones  (Fig.  80),  especially  those  of  the 
feet   (Figs.   81,   82),  is  often  obvious  during  life.     The 


Fig.  82.— Skiagram  showing  very  advanced  atrophy  of  the  hones 
of  the  foot.    (From  a  case  under  the  care  of  Mr.  T.  H.  KeUcck.)- 


big  toe  is  very  frequently  shortened,  owing,  as  shown 
by  the  X-rays,  to  wasting  of  its  bones.  In  the  feet 
and,  more  rarely,  in  the  vertebrae  the  bones  and  joints 
are  apt  to  show  changes  at  the  same  time. 

The    crises.  —  Paroxysmal    disturbances    of    function 
(known  as  crises)  are  apt  to  occur  in  connection  with 


300    DISEASES   OF   THE  NERVOUS   SYSTEM 

the  various  viscera.  In  their  production  they  apj)ear 
to  be  analogous  to  the  lightning  pains,  and  for  the  most 
part  they  probably  depend  upon  degenerative  changes 
in  the  sympathetic  system. 

Gastric  crises. — These  form  the  commonest  variety  of 
crises.  Every  few  weeks  the  patient  has  attacks  of  in- 
tense pain  and  sickness,  which  for  the  time  being  entirely 
incapacitate  him  from  work.  The  duration  of  these 
attacks  varies  from  a  few  days  to  a  week  or  more,  and 
in  the  intervals  there  may  be  no  symptoms  whatever. 

Wilfred  Harris  has  observed  the  occasional  occur- 
rence of  epileptiform  attacks  during  the  gastric  crises, 
and  considers  that  there  is  some  causal  relationship 
between  the  two  conditions. 

Laryngeal  crises. — In  these  the  patient  gets  signs  of 
spasm  of  the  laryngeal  muscles,  accompanied  by  a  bark- 
ing and  crowing  cough  which  in  some  ways  resembles 
whooping-cough. 

Other  organs  subject  to  crises  are  the  heart,  bladder, 
rectum,  and  kidneys. 

Ocular  symptoms. — The  Argyll- Robertson  pupil  is  one 
of  the  commonest  symptoms  of  tabes  dorsalis.  Its 
characteristic  is  loss  of  reaction  to  light,  while  contrac- 
tion during  accommodation  remains.  (Fig.  83.)  The 
pupils  are  generally  very  small  (pin-point),  often  unequal, 
and  frequently  show  some  loss  of  that  perfect  rotundity 
by  which  they  are  distinguished  in  health. 

The  Argyll-Robertson  pupil  is  often  the  earliest  sign 
of  tabes,  and  it  may  occasionally  be  observed  to  exist 
for  a  time  in  one  eye  only.  When  the  degeneration  is 
more  extensive  the  contraction  during  accommodation 
may  also  be  absent. 

The  lesion  causing  the  loss  of  light  reflex  in  the 
Argyll-Robertson  pupil  is  possibly  situated  in  the  fibres 
which  complete  the  reflex  arc  by  joining  the  optic  nerve 
to  the  nucleus  of  the  3rd  nerve  (Fig.  83).  In  a  case 
in  which  there  is  no  question  of  optic  atrophy,  it  is 
obvious  that  the  fibres  of  the  optic  nerve  are  healthy,  and 
in  like  manner  the  ability  of  the  pupil  to  contract  in 


TABES   DORSALIS  301 

association  with  accommodation  shows  that  impulses 
can  pass  freely  along  the  fibres  of  the  3rd  nerve.  The 
inference,  therefore,  is  that  the  degeneration  in  such  a 
case  has  taken  place  in  the  fibres  between  the  two  nerves. 
Another  view  is  that  the  Argyll-Robertson  pupil  is 
caused  by  degeneration  of  the  ciliary  ganglion. 


Fig.  83.— Diagram  of  the  pupil  reflex  to  light.    (WilfreclHarris.) 


Optic  atro'pJiy. — ^A  primary  atrophy  of  the  optic 
nerve  is  apt  to  occur  in  the  course  of  locomotor  ataxy, 
and  sometimes  failure  of  sight  from  this  cause  is  the 
first  symptom  to  attract  the  patient's  attention.  The 
disc  assumes  a  pearly-white  appearance,  and  the  vessels 
running  over  it  are  relatively  small.  The  rate  at  which 
sight  is  lost  varies  greatly,  and  it  may  be  some  three, 
four,  five,  or  more  years  before  anything  like  complete 
blindness  supervenes. 


302    DISEASES   OF   THE   NERVOUS   SYSTEM 

Paresis  of  muscles. — Paresis  of  the  ocular  muscles 
indicates  a  lesion  of  the  motor  neurones,  and  it  is  in 
this  region  that  the  motor  symptoms  in  tabes  are  most 
commonly  found. 

Any  of  the  ocular  muscles  may  be  affected,  but  the 
external  rectus  is  the  one  most  prone  to  suffer.  Squint 
and  diplopia  are  the  result,  but  as  a  rule  the  paresis 
is  only  of  temporary  duration,  and  it  is  common  for 
patients  to  complain  from  time  to  time  of  double  vision, 
which  completely  passes  aAvay  in  the  intervals. 

Much  less  common,  but  more  important,  are  the  per- 
manent paralyses  in  which  all  the  branches  of  the  3rd 
nerve  are  liable  to  be  affected,  and  which  are  probably 
due  to  degeneration  of  the  motor  nuclei. 

Ptosis  is  sometimes  temporary,  thus  resembling 
the  transient  paralysis  of  other  ocular  muscles,  but 
at  other  times  it  may  be  permanent  and  be  part  of  a 
complete  paralysis  of  the  3rd  nerve  (Fig.  84). 

Other  cranial  nerves. — No  cranial  nerves  are  exempt 
from  the  possibility  of  degeneration  ;  the  olfactory  and 
auditory  nerves  may  suffer,  and  sometimes  there  are 
signs  of  degeneration  of  the  nuclei  in  the  medulla. 
Anaesthesia  and  pains  not  infrequently  occur  over  the 
regions  supplied  by  the  5th  nerve. 

Diacjiiosis. — In  considering  the  diagnosis  of  loco- 
motor ataxy  it  is  important  to  remember  the  possibility 
of  the  signs  appearing  in  almost  any  order.  In  one  case 
the  pains  may  be  the  most  prominent  symptom  ;  in 
another  a  joint  may  be  the  chief  cause  of  anxiety ;  in 
a  third  it  may  be  the  eyes  that  first  give  rise  to  trouble  ; 
and  in  yet  another  there  may  be  difficulty  in  exercising 
full  command  over  the  bladder.  It  is  most  necessary 
in  diagnosing  tabes  to  remember  that  undue  stress  must 
not  be  laid  upon  the  absence  of  one  or  more  signs  that 
ought  to  be  present  in  a  "  typical  "  case.  For  instance, 
because  the  knec-jei'k  is  usually  lost  by  the  time  most 
patients  come  for  diagnosis,  its  presence,  and  some- 
times even  its  increase,  must  not  be  allowed  to  negative 
the  possibility  of  tabes  if  other  signs  point  to  such  a 


TABES  DORSALIS:   DIAGNOSIS  303 

conclusion.  When  the  roots  are  affected  in  such  a 
variable  order  as  they  are  in  tabes,  it  is  no  wonder  if 
those  upon  which  the  presence  of  the  knee-jerks  depends 
are  not  always  among  the  first  to  degenerate. 

The  presence  of  Argyll-Robertson  pupils  in  associa- 
tion with  any  other  suspicious  symptom — e.g.  lightning 


Fig.  84.— Case  of  tabetic  ptosis,  showing  the  com- 
pensatory contraction  of  the  occipito-frontalis. 

pains,  bladder  trouble,  or  a  swollen  joint  of  doubtful 
nature — ^is  practically  pathognomonic  of  tabes,  pro- 
vided that  the  onset  of  general  paralysis  of  the  insane 
can  be  excluded. 

It  occasionally  happens  that,  during  the  routine 
examination  of  patients,  Argyll-Robertson  pupils  are 
discovered    as   isolated    signs   and    are    associated   with 


301    DISEASES   OF   THE   NERVOUS   SYSTEM 

no  other  detectable  morbid  conditions  whatever.  The 
question  naturally  then  arises  as  to  what  interpretation 
should  be  put  upon  such  a  case.  On  the  whole  it  would 
seem  that  the  most  reasonable  attitude  to  adopt  in 
such  cases  is  to  look  upon  the  sign  as  evidence  of  local 
degeneration,  probably  of  a  parenchymatous  syphilitic 
nature  ;  but  experience  shows  that,  although  its  presence 
is  very  suspicious,  it  may  nevertheless  exist  for  an 
indefinite  time  without  signs  of  degeneration  of  the 
nervous  system  appearing  elsewhere. 

Peripheral  neuritis. — Cases  of  peripheral  neuritis 
in  which  sensory  symptoms  are  prominent  sometimes 
resemble  tabes  in  many  respects,  for  common  to  the  two 
disorders  may  be  pains,  loss  of  knee-jerks,  and  incoordina- 
tion, and  occasionally  the  similarity  is  increased  by  the 
presence  of  sores  on  the  feet,  which  may  be  mistaken 
for  "  perforating  ulcers."  The  closest  similarity  seems 
to  occur  in  some  of  the  cases  of  neuritis  due  to  alcohol 
and  to  diabetes,  but  a  systematic  examination  generally 
removes  the  doubt  by  revealing  j)ositive  signs  of  one  or 
other  disease.  An  Argyll-Eobertson  pu23il,  for  instance, 
would  be  conclusive  evidence  in  favour  of  tabes,  while 
loss  of  power  and  electrical  changes  would  strongly 
support  the  sujoposition  of  neuritis. 

Friedreich's  disease  has  several  symptoms  in  com- 
mon with  tabes — e.g.  loss  of  tendon  reflexes  and  inco- 
ordination— but  the  age  of  onset  and  the  family  nature 
of  the  disease,  together  with  the  speech  defects,  nodding 
movements,  and  nystagmus,  usually  serve  to  distinguish 
it,  though,  as  far  as  age  is  concerned,  it  must  be  re- 
membered that  cases  of  juvenile  tabes  may  occur. 

Ataxic  paraplegia. — The  combination  of  spasticity 
with  incoordination,  together  with  the  increased  knee- 
jerks  and  the  absence  of  eye  symptoms,  will  serve  to 
distinguish  ataxic  paraplegia  from  tabes. 

Cerebellar  disease. — The  ataxy  of  cerebellar  disease 
does  not  usually  become  distinctly  worse  when  the  eyes 
are  closed. 

If  there  is  a  tumour  the  history  and  symptoms  will 


TABES   DORSALIS  :    PROGNOSIS  305 

not  be  suggestive  of  tabes,  and  if  the  ataxy  is  due  to 
cerebellar  deficiency  the  history  of  the  case  will  enable 
tabes  to  be  excluded. 

Disseminated  sclerosis. — Disseminated  sclerosis  ap- 
pears in  so  many  forms  that  in  some  of  them  it  may 
occasionally  resemble  tabes,  but,  even  in  those  instances 
where  confusion  is  likely  to  arise,  exaggerated  knee- 
jerks  (loss  of  knee-jerks  is  very  uncommon  in  dis- 
seminated sclerosis),  alteration  in  speech,  nystagmus, 
and  tremors  on  movement  are  generally  present  in  some 
degree. 

Interstitial  spinal  syphilis. — Syphilitic  disease  of  the 
membranes,  causing  secondary  changes  in  the  posterior 
roots,  combined  as  it  may  be  with  thrombosis  of  vessels 
in  the  posterior  columns  of  the  cord,  may  give  rise  to 
signs  which  are  in  some  ways  almost  indistinguishable 
from  those  of  tabes. 

Pain  in  the  back,  loss  of  power  (of  flaccid  type,  due 
to  implication  of  anterior  roots,  or  of  spastic  type,  due 
to  extension  of  the  thrombotic  processes  into  the  lateral 
columns),  and  the  variability  of  the  symptoms  are 
among  the  distinguishing  features.  The  presence  of  co- 
existing signs  of  interstitial  cerebral  syphilis  often 
assists  in  making  the  diagnosis,  but  it  must  be  re- 
membered that  parenchymatous  and  interstitial  disea^'e 
of  the  brain  and  cord  may  exist  at  the  same  time. 

Prognosis. — Probably  in  no  other  chronic  disease 
of  the  spinal  cord  does  degeneration  vary  more  in  its 
rate  of  progress  than  it  does  in  tabes. 

After  the  appearance  of  the  first  symptoms,  many 
years  often  elapse  before  there  are  any  signs  of  ataxy. 
The  symptoms  may  cease  to  progress  at  various  stages 
of  the  disease,  and  in  a  certain  number  of  cases  some 
definite  improvement  occurs.  In  these  circumstances 
it  will  be  obvious  how  important  it  is  to  try  to  form  an 
estimate  of  the  probable  future  of  every  case  in  order 
to  be  in  a  position  to  give  some  practical  advice  regarding 
the  best  course  for  the  patient  to  pursue. 

This,  as  far  as  is  possible,  may  be  accomplished  by 
V 


3v06    DISEASES   OF   THE   NERVOUS   SYSTEM 

paying  attention  to  tlie  patient's  age  and  circumstances, 
the  rate  at  which  the  degeneration  has  progressed,  and 
the  position  of  the  first  signs  of  degeneration. 

It  has  been  observed  that  the  course  of  tabes  is, 
as  a  rule,  much  slower  when  it  begins  in  elderly  people 
of  50  or  60  than  when  it  attacks  those  of  younger  years, 
e.g.  from  25  to  30.  In  these  latter  the  degeneration  often 
proceeds  very  rapidly. 

The  circumstances  of  the  patient  must  be  taken 
into  account  in  forming  a  prognosis,  since  those  who 
have  money  and  leisure  are  in  a  better  position  to  combat 
the  disease  than  those  who  are  obliged  to  continue  their 
daily  work. 

if  at  the  time  of  observation  the  patient  gives  a 
history  of  tabetic  symptoms  existing  over  a  period 
of  some  years,  and  examination  shows  that  the  extent 
of  the  degenerative  processes  is  still  very  limited,  it  is 
reasonable  to  infer,  with  certain  reservations,  that  the 
changes  will  continue  to  proceed  slowly. 

In  this  connection  Sir  William  Gowers  directed  at- 
tention to  a  group  of  cases  in  which,  though  lightning 
pains  have  existed  for  a  number  of  years,  there  is  no 
loss  of  knee-jerks  and  no  sign  of  atax}^  According  to 
Gowers,  these  should  be  considered  as  a  special  variety 
of  tabes  depending  upon  seme  peculiarity  of  the  toxin 
rather  than  ordinary  cases  in  which  development  of  the 
disease  has  been  abnormally  slow.  The  future  of  such 
cases  appears  to  be  comparatively  favourable,  and 
until  the  knee-jerks  are  lost  no  ataxy  is  likely  to  arise. 

It  is  also  well  known  that  patients  who  develop 
optic  atrophy  early  often  remain  for  a  long  time  without 
any  signs  of  ataxy. 

In  predicting  a  slow  degeneration  for  the  future  on 
the  strength  of  one  that  has  been  slow  in  the  past,  it 
must  not  be  forgotten  that  very  rapid  increase  in  the 
intensity  of  symptoms  is  sometimes  brought  about  by 
shock,  illness,  exposure  to  cold,  or  injuries. 

Trcalmciit. — The  difficulty  in  obtaining  favour- 
able results  from  the  administration  of  mercury  and 


TABES    DORSALIS:    TREATMENT         307 

salvarsan  in  cases  of  parencliymatons  syphilis  lias  already 
been  empliasized,  and  up  to  tlie  present  there  is  little 
evidence  of  any  striking  benefit  having  been  produced  by 
these  methods  of  treatment.  Preparations  of  salvarsan 
given  intravenously  or  intrathecally  in  the  form  of  im- 
munized serum  appear  in  some  instances  to  have  been 
followed  by  relief  of  lightning  pains  and  some  general 
improvement,  but  there  does  not  seem  to  be  any  con- 
clusive evidence  that  the  progress  of  the  disease  can 
be  permanently  arrested  by  this  method.  It  appears 
to  be  likely,  however,  that  more  benefit  may  be  obtained 
by  systematic  treatment  in  the  case  of  tabes  than  in 
general  paralysis,  since  the  progress  of  tabes  is  so  much 
slower,  and  frequently  becomes  more  or  less  stationary; 
and  it  is  usually  considered  advisable  for  the  patient  to 
undergo  a  course  of  antisyphilitic  treatment  from  time 
to  time  as  a  precautionary  measure  against  further 
extension  of  tke  disease.  If  the  hypothesis  mentioned 
above  (p.  286),  that  in  some  cases  the  mischief  is  pro- 
duced by  a  proteolytic  ferment  which  has  its  origin 
externally  to  the  cord,  should  prove  to  be  correct,  it 
would  seem  that  the  antidote  should  have  a  better 
chance  of  reaching  the  focus  of  infection  than  in  cases 
where  the  trouble  is  due  to  activity  of  the  spirochaete 
within  the  cord  ;  hence,  for  the  present  it  will  be  well 
to  keep  an  open  mind  on  the  efiects  of  treatment  in  cases 
that  have  been  carefully  selected  as  likely  to  receive 
benefit. 

Apart  from  antisyphilitic  remedies,  reliance  must  be 
placed  on  increasing  the  resisting  powers  of  the  patient 
by  improving  his  nutrition,  which  can  best  be  done 
by  prescribing  suitable  tonics,  with  a  proper  amount 
of  food  and  rest. 

Of  tonics,  the  author  places  most  reliance  on  iron, 
and  it  seems  reasonable  to  suppose  that  an  improve- 
ment in  tbe  quality  of  the  blood  will  be  followed  by  an 
improvement  in  the  nutrition  of  the  nerve  fibres.  The 
perchloride,  in  doses  of  10  to  15  minims  three  times  a 
day,  appears  to  answer  best,  but,  if  it  disagrees,  other 


308    DISEASES   OF   THE   NERVOUS   SYSTEM 

preparations  may  be  substituted.  If  it  be  thought  ad- 
visable, arsenic  may  be  given  with  the  iron.  Aluminium 
chloride  was  introduced  into  therapeutics  by  Sir  William 
Gowers  for  its  apparent  usefulness  in  modifying  the 
intensity  and  frequency  of  the  lightning  pains,  and 
it  also  may  be  given  in  combination  with  the  iron  and 
arsenic  in  some  such  prescription  as  the  following  : — 

^  Tinctura3  ferri  perchloridi       ...       Dl,     7  ...  iiixii 

Liquoris  arsenici  hydrochlorici       CI.   18  ...  luiii 

Aluminii  chloridi          ...         ...       Dg.   2  ...  gr.  iii 

Spiritus  chlorofoi'mi    ...         ...       Ml.    1  ...  iilxv 

Aqiiam ad  Ml.  30  ...  ^i 

Two  tablespoonfuls  to  be  taken  three  times  a  day  after  meals. 

Strychnine  is  a  drug  which  may  also  occasionally 
be  given  with  benefit. 

Plenty  of  nourishing  food  should  be  taken,  for  the 
nutrition  of  these  patients  is  frequently  poor,  and,  in 
many  instances,  cod-liver  oil  is  useful. 

Rest  is  an  important  factor  in  the  course  of  treat- 
ment. The  degeneration  seemingly  has  a  tendency  to 
occur  in  those  nerves  whose  functions  are  most  hardly 
used,  and  to  continue  to  exercise  these  nerves  as  much 
as  before  is  only  to  court  an  extension  of  the  degenerative 
processes. 

Walking  (for  the  disease  generally  begins  in  the 
roots  at  the  lower  part  of  the  cord)  should  be  slow, 
the  distances  should  be  short,  and,  whenever  possible, 
driving  should  be  substituted.  Fatigue  both  of  mind 
and  body  should  be  avoided  as  much  as  possible,  and 
it  is  scarcely  necessary  to  add  that  plenty  of  fresh  air, 
within  doors  as  well  as  without,  is  essential. 

The  various  forms  of  treatment  by  electricity  have, 
at  most,  nothing  more  than  a  passing  tonic  effect. 

To  restore  the  functions  of  those  fibres  which  are 
already  dead  is,  of  course,  impossible,  but  in  suitable 
circumstances,  and  by  careful  attention  to  detail,  there 
is  no  doubt  that  something  can  be  done  to  retard  the 
extension  of  degeneration. 


TABES   DORSALLS:    TREATMENT         309 

It  must  also  be  recollected  that  in  disease,  just  as 
in  health,  function  is  often  a  degree  above  or  below 
what  may  be  the  average  level  for  any  particular  person. 
Tabetic  patients  with  some  degree  of  optic  atrophy  can 
see  much  better  on  some  days  than  others  ;  so,  also,  those 
with  ataxy  can  walk  better  on  some  days  than  others. 
To  keep  these  patients  at  their  highest  possible  level  of 
health  should  be  one  of  the  objects  of  treatment. 

Treatment  of  lightning  pains. — To  relieve  the  light, 
ning  pains  of  tabes  is  often  a  difficult  matter.  Among 
the  most  reliable  drugs  are  phenacetin,  antipyrin, 
antifebrin,  and  aspirin ;  sometimes  success  is  obtained 
wdth  one  and  sometimes  with  another,  and  occasionally 
a  combination  is  very  effective.  They  may  be  given 
conveniently  in  cachets,  and,  when  the  attack  is  very 
severe,  Tourette  has  recommended  10  minims  of  tincture 
of  opium  after  each  cachet,  provided  that  not  more  than 
30  or  40  minims  are  given  in  twenty-four  hours.  Careful 
attention  should  be  paid  to  the  state  of  the  alimentary 
canal,  as  constipation  often  appears  to  be  a  determining 
factor  in  bringing  on  pains. 

Injections  of  morphia  will,  of  course,  give  relief, 
but,  on  account  of  the  danger  of  the  patient  acquiring 
a  craving  for  this  drug,  they  should  only  be  resorted  to 
in  very  exceptional  cases. 

The  various  crises  must  be  treated  by  similar  methods. 
In  the  gastric  crises  the  difficulty  of  retaining  drugs  in  the 
stomach  sometimes  makes  it  necessary  to  give  relief  by 
hypodermic  injections  or  by  rectal  suppositories.  In 
very  severe  cases  division  of  the  posterior  roots  of  the 
nerves  supplying  the  stomacli  (Ttb-llth  dorsal)  has 
been  occasionally  practised,  but  it  is  an  operation  of 
considerable  severity,  and  uncertain  in  its  results. 

Treatment  of  bladder  troubles. — When  it  is  necessary 
to  draw  off  the  urine  the  utmost  care  must  be  taken 
to  use  an  aseptic  catheter.  When  cystitis  has  occurred, 
if  the  urine  is  acid,  urotropine  with  or  without  acid 
sodium  phosphate  may  be  given ;  if  alkaline,  boric 
acid,  ammonium  benzoate,  and  acid  sodium  phosphate 


310    DISEASES   OF   THE   NERVOUS   SYSTEM 

should  Ijc  used.  If  necessary,  the  bladder  must  be 
washed  out. 

Treatment  of  ataxy. — To  many  patients  the  inability 
to  walk  straight  is  their  most  distressing  symptom, 
and  the  methods  introduced  by  Frenkel  for  correcting 
this  difficulty  by  means  of  systematic  exercises  have 
proved  to  be  of  decided  value. 

Incoordination  is  the  result  of  degeneration  of  nerve 
fibres  from  the  muscles,  joints,  and  ligaments  whereby 
the  sense  of  position  of  the  limbs  is  greatly  diminished, 
often  so  much  so  that  when  the  eyes  are  closed  the 
patient  is  unable  to  tell  at  what  angle  his  arms  or  legs 
are  flexed. 

The  object  of  Frenkel's  exercises  is  to  improve  the 
sensibility  of  the  remaining  fibres  so  that  their  in- 
creased function  will  fill  the  gap  left  by  those  that  are 
degenerated. 

In  an  average  person  the  functions  of  the  special 
senses  are  seldom,  if  ever,  educated  to  the  highest 
possible  pitch — a  fact  which  is  clearly  shown  by  the 
delicate  sensibility  for  touch  and  sound  which  may  be 
acquired  by  the  blind.  In  tabes,  unless  the  degenera- 
tion is  exceptionally  extensive,  there  is  a  considerable 
margin  left  for  the  possibility  of  improving  the  muscle- 
sense,  and  by  taking  advantage  of  this  possibility  the 
incoordination  can  to  a  great  extent  be  corrected. 

To  obtain  success,  the  exercises  must  be  performed 
slowdy  and  accurately,  great  care  being  taken  to  avoid 
fatigue,  which,  as  stated  above,  is  so  harmful.  It  is 
necessary  to  insist  upon  this  point,  because  the  efforts 
made  to  move  the  limbs  slowly  and  accurately  are  a 
great  strain  on  the  mind  as  well  as  on  the  body. 

Various  exercises  can  be  devised  without  much  trouble 
and  expense.  The  patient  may  be  taught  to  regain 
command  over  his  legs  by  lying  on  a  couch  and  raising 
the  foot  from  square  to  square  of  a  wooden  frame  so 
that  the  big  toe  is  brought  to  rest  smoothly  at  each 
movement,  and  moving  the  foot  backwiirds  and  forwards 
so  that  the  heel  is  placed  accurately  on  various  marked 


TABES   DORSALLS:    TREATMENT         311 

spots.  To  this  may  ha  added  exercises  in  walking 
accurately  to  patterns  on  the  floor ;  while,  later  on, 
walking  sideways  and  other  more  difficult  feats  may 
be  attempted. 

The  hands  may  be  trained  by  means  of  draught 
and  halma  boards,  or  some  similar  objects,  by  which 
accuracy  and  attention  are  brought  to  bear  on  the 
movements. 

Schwab  and  Allison  *  have  shown  that  in  tabes  the 
foot  tends  to  assuine  a  faulty  position  of  eversion,  and 
that  by  correcting  this  with  suitable  boots  a  mechanical 
advantage  is  obtained  which  further  assists  to  diminish 
the  incoordination. 

*  Journ.  of  Amer,  lied.  Assoc,  Dec.  16,  1905. 


SECTION  VI. -DISEASES  OF  THE 
BRAIN,  AND  DISEASES  OF  GEN- 
ERAL ORIGIN 

CHAPTER  XXXIV 
MENINGITIS 

Inflammation  of  the  membranes  cf  the  brain  may 
occur  as  part  of  a  general  infective  disorder,  cr  it  may 
arise  from  extension  of  some  local  disease  of  the  skull. 
In  the  latter  case  the  dura  mater  is  especially  liable 
to  be  affected  {pachymeningitis),  but  in  the  former  the 
primary  seat  of  the  inflammation  is  generally  in  the  pia 
mater  and  arachnoid  (leptomeningitis). 

The  micro-organisms  which  most  frequently  invade 
the  meninges  are  those  of  tuberculosis,  pneumonia, 
typhoid,  septicaemia,  cerebro-spinal  fever  and  its  sporadic 
form  posterior  basic  meningitis.  In  many  cases  the 
primary  infective  organism  becomes  associated  with 
streptococci  and  staphylococci  during  the  course  of  the 
illness. 

While  the  invasion  of  the  meninges  may  be  the 
predominating  feature  during  life,  an  examination  of 
the  body  after  death  very  frequently  reveals  disease  in 
other  organs  as  well.  Thus,  tuberculous  meningitis  is 
practically  always  a  part  of  a  more  generalized  infection, 
as  also  are  the  cases  due  to  the  organisms  of  typhoid  and, 
though  perhaps  less  often,  of  pneumonia.  The  syphilitic 
forms  have  been  already  mentioned  (Section  V.). 

I.  Tuberculous  Meningitis 
Etiology. — Tuberculous  meningitis  is  most  commonly 
found  in  children  from  2  to  10  years  of  age,  though  its 
occurrence  in  adults  is  by  no  means  rare. 

312 


TUBERCULOUS   MENINGITIS  313 

The  contributory  causes  are  debility  and  the  presence 
of  tuberculosis  of  the  lungs,  glands,  or  some  other  part 
of  the  body. 

Symptoms. — The  early  symptoms  vary  according 
to  (1)  the  intensity  of  the  general  toxaemia,  and  (2) 
the  prominence,  or  otherwise,  of  local  symptoms. 

As  a  rule,  there  are  signs  of  general  disturbance  for 
some  time  before  there  is  any  reliable  evidence  of  cerebral 
trouble,  the  child  being  irritable,  languid,  and  generally 
out  of  sorts.  An  early  sign  of  considerable  importance 
is  vomiting  for  which  no  adequate  cause  can  be  found, 
and  with  which  is  often  associated  constipation.  The 
temperature  is  a  little  raised  at  night,  and  often  runs  an 
irregular  course  throughout  the  illness. 

In  other  cases,  distinct  cerebral  signs  occur  at  an 
earlier  date,  and  severe  headache,  with  perhaps  a  squint 
and  a  general  convulsion,  may  be  among  the  symptoms 
by  which  attention  is  first  attracted,  though  here  again 
careful  inquiry  often  elicits  the  fact  that  the  child 
has  not  for  some  time  previously  been  in  its  usual 
health. 

The  symptoms  can  best  be  remembered  by  consider- 
ing what  effects  the  pathological  changes  produce  upon 
the  various  parts  of  the  brain.  The  small,  grey,  semi- 
translucent  tubercles  are  situated  in  the  pia  mater  and 
are  distributed  chiefly  over  the  base  of  the  brain.  They 
are  very  prone  to  occur  along  the  course  of  the  middle 
cerebral  artery,  and  are  generally  most  easily  recognized 
between  the  opercula  of  the  Sylvian  fissure.  They 
are  usually  much  less  numerous  over  the  convexity  of 
the  brain,  but  in  this  respect  their  distribution  is  very 
variable  in  different  cases. 

Their  growth  is  accompanied  by  a  serous  exudate 
of  varying  density.  The  chief  effects  produced  by 
these  processes  are  : 

1.  Irritation  and  compression  of  different  structures 
of  the  brain. 

2.  General  rise  of  intracranial  pressure  due  to  the 
exudation  of  fluid  and  the  hmitation  of  its  circulation 


3U    DISEASES   OF   THE   NERVOUS   SYSTEM 

by  the  adlie.siou  of  the  infiamcd  parts,  and  cspecia'.ly 
by  the  blocking  of  the  foramen  of  Magendie  and  the 
consequent  distension  of  the  cerebral  ventricles. 

The  local  effects  of  the  irritation  and  compression 
are  chiefly  felt  by  the  cranial  nerves,  and  may  be  roughly 
enumerated  as  follows  : 

Olfactory  nerve. — Usually  no  definite  signs  of  im- 
pairment. 

Ovtic  nerve. — Optic  neuritis  is  common,  and  a  useful 
aid  to  diagnosis.  During  an  examination  of  the  eyes 
for  evidence  of  optic  neuritis  the  possible  presence  of 
tubercles  in  the  choroid  should  be  remembered. 

Third,  fourth,  and  sixth  nerves. — These  three  motor 
nerves  of  the  eye  may  be  considered  together.  One 
or  more  are  often  affected,  with  resulting  squint  and 
ptosis.  The  pupils  may  be  unequal,  and  reaction  to 
light  lost  or  impaired. 

Trigeminal  nerve. — There  are  not  usually  any  definite 
signs  connected  with  the  5th  nerve,  but  if  present  they 
will  take  the  form  of  pain  in  the  area  of  its  surface 
distribution  and  of  spasm  or  paresis  of  the  muscles  of 
mastication. 

Facial  nerve. — Extension  of  the  inflammation  to  the 
trunk  of  this  nerve  gives  rise  to  weakness  of  a  part  or 
the  whole  of  one  side  of  the  face.  Twitching  of  the 
facial  muscles  may  also  occur  if  irritation  be  present. 

Auditory  nerve. — ^Deafness  results  from  implication 
of  the  auditory  nerve.  It  is  not  usually  a  prominent 
symptom,  for  the  impaired  mental  condition  of  the 
patient  generally  overshadows  it. 

Glosso-fharyngeal  nerve. — There  is  no  obvious  symp- 
tom connected  with  the  glosso-pharyngeal. 

Vagus. — During  certain  stages  of  the  disease  irrita- 
tion of  the  vagus  may  show  itself  by  the  slow  beating 
of  the  heart,  but  paralytic  signs  are  usually  the  more 
prominent,  and  the  heart  becomes  rapid  and  irregular. 
The  respiratory  rhythm  is  also  altered,  but  the  functions 
of  both  heart  and  respiration  are  in  all  probability 
largely  modified  by  the  more  general  effects  of  the  disease 


TUBERCULOUS   MENINGITIS  315 

upon  other  jxirts  of  the  brain,  and  tlic  variations  of 
the  respiratory  rhythm  will  be  again  considered  under 
that  heading. 

Sfinal  accessory  nerve. — Irritation  of  the  spinal 
portion  of  the  spinal  accessory  is  perhaps  responsible 
to  some  extent  for  the  retraction  of  the  head,  though 
probably  this  is  also  influenced  by  irritation  of  the 
cerebellum ;  but  retraction  of  the  head  is  often  tempo- 
rary, and  in  most  cases  is  not  a  prominent  feature  of 
tuberculous  meningitis,  which  is  consistent  with  the  fact 
that  the  exudation  is  frequently  limited  chiefly  to  the 
area  of  the  circle  of  Willis,  and  does  not  extend  suffi- 
ciently far  down  to  implicate  the  cerebellum  and  the 
cervical  nerve  roots. 

Hypoglossal  nerve. — There  are  not  usually  any  signs  of 
disturbance  of  the  function  of  the  hypoglossal  nerve. 

The  convexity  of  the  brain. — When  the  inflammation 
extends  on  to  the  convexity  of  the  brain  there  may 
be  monoplegia  or  hemiplegia.  LocaHzed  convulsions 
may  also  be  a  prominent  symptom. 

When  we  come  to  consider  the  effect  of  the  general 
increase  of  intracranial  pressure,  we  find  a  number  of 
symptoms  referable  to  compression  and  irritation  of  the 
cortex  and  meninges. 

Pain. — The  headache,  perhaps  slight  in  the  early 
stages,  may  become  so  intense  as  to  cause  the  inter- 
mittent shrieking  known  as  the  "  hydrocephalic  cry." 

Vomiting. — Early  in  the  disease  the  vomiting  may 
be  due  to  the  general  toxaemia,  but  in  the  later  stages 
it  is  mainly  the  result  of  the  rise  of  intracranial  pressure, 
as  in  the  case  of  cerebral  tumours. 

The  respiratory  rhythm  is  altered.  Sometimes  it  is 
of  the  Cheyne-Stokes  type,  but  more  often  a  few  respira- 
tions of  equal  intensity  are  succeeded  by  a  short  pause. 
The  breathing  is  nearly  always  rapid. 

The  pulse  for  the  most  part  is  quickened  ana  irregular, 
but  in  some  stages  of  the  illness  it  may  be  slow  from 
irritation  of  the  vagus,  as  already  mentioned. 

Convulsions  are  not  uncommon.     They  arise  from 


316    DISEASES   OF   THE   NERVOUS   SYSTEM 

extension  of  the  tubercles  on  to  the  cerebral  cortex  or 
from  irritation  induced  by  the  general  rise  of  pressure  ; 
localized  twitchings  also  frequently  occur. 

The  abdomen  is  retracted,  and  known  as  the  "  scaphoid 
abdomen." 

There  are  pronounced  mental  symptoms  ;  the  patient 
is  irritable,  lies  curled  up  on  one  side,  avoids  the  light, 
and  shows  disinclination  to  answer  questions  or  to  be 
troubled  in  any  way. 

The  vaso-motor  system  is  deranged,  and  if  the  finger- 
nail be  drawn  over  the  skin  it  is  rapidly  followed  by  a 
flush  that  is  known  as  the  tache  cerebrale. 


Fig.  85.— Method  of  obtaining  Kernig's  sign. 
(After  a  photograph  by  Dr.  W.  Thyne.) 

The  temperature  throughout  the  illness  is  somewhat 
irregular,  and  not  generally  very  high  ;  but  towards 
the  end  there  is  often  a  considerable  rise,  which  may 
continue  for  some  time  after  death. 

Kernic/s  sign. — The  effects  of  cerebral  irritation  are 
seen  in  the  increased  "  tone  "  (hypertonus)  of  muscles, 
and  it  is  on  this  fact  that  Kernig's  sign  depends  (Fig.  85). 
To  obtain  the  sign  the  patient  should  be  lying  on  the 
back,  and,  care  being  taken  to  keep  one  leg  flat  on  the 
bed,  the  other  should  be  flexed  at  the  hip  and  knee. 
When  the  tone  of  the  muscles  is  increased,  the  degree  of 


TUBERCULOUS    MENINGITIS  317 

extension  wliich  it  is  possible  to  obtain  at  the  knee-joint 
while  the  thigh  is  flexed  upon  the  abdomen  is  con- 
siderably less  than  normal.  Kernig's  sign  is  said  to  be 
present  in  over  80  per  cent,  of  the  cases  of  meningitis. 
It  is,  therefore,  of  considerable  diagnostic  significance, 
but  it  may,  of  course,  be  associated  with  other  forms 
of  cerebral  irritation. 

General  course  ol  the  disease. — ^Attempts  have 
been  made  to  divide  the  disease  into  stages  of  irritation 
and  compression,  but  the  two  conditions  are  necessarily 
concurrent,  and  it  is  very  seldom  that  any  sharp  line 
can  be  drawn  between  them. 

The  duration  of  the  symptoms  is  very  variable. 
Death  may  occur  within  a  week  or  ten  days  from  the 
onset  of  acute  symptoms,  or  it  may  be  postponed  for 
three  weeks  or  more. 

Prognosis. — Though  cases  have  occasionally  been 
recorded  in  which  recovery  has  taken  place  from  symp- 
toms that  seem  to  have  been  undoubtedly  due  to 
meningitis  of  tuberculous  origin,  such  instances  are  so 
rare  that  for  practical  purposes  the  disease  must  be 
regarded  as  one  which  is  always  likely  to  cause  death. 
Moreover,  as  already  insisted  upon,  the  meningeal 
inflammation  is  often  only  the  more  prominent  part  of 
a  widely  spread  infection. 

Diagnosis. — In  children  the  chief  difficulty  in 
arriving  at  a  diagnosis  exists  during  the  early  stages 
of  the  disease.  The  child  has  perhaps  been  ailing  for 
some  time,  but  gets  worse  so  gradually  that  it  is  difficult 
to  assign  the  correct  value  to  the  vague  symptoms  of 
headache  and  gastric  disturbance  which  so  often  precede 
those  of  a  more  definite  character. 

In  other  cases  the  difficulty  is  of  the  opposite  kind, 
for  the  symptoms  come  on  so  acutely  that  for  a  short 
time  it  appears  more  likely  that  an  acute  specific  fever 
is  about  to  develop.  Between  these  two  extremes  there 
is  every  gradation,  so  that  it  is  frequently  difficult  to 
make  a  diagnosis  with  any  certainty  at  an  early  stage 
of  the  disease. 


318    DISEASES   OF   THE   NERVOUS   SYSTEM 

Inflammatory  troubles  cf  the  middle  ear,  the  mastoid, 
and  other  parts  of  the  skull  may  for  a  time  be  mistaken 
for  meningitis,  as  also  may  the  mental  disturbance 
sometimes  due  to  intesthial  worms. 

In  adults  the  distinction  between  typhoid  fever  and 
tuberculous  meningitis  is  often  difficult.  The  main 
points  to  be  relied  upon  can  be  tabulated  as  follows  : 


Typhoid 

Meningitis 

Headache  ... 

Optic  neuritis 
Ehrliclt^s  reaction... 

Early      symptom  ; 
tends  to  disappear 
later 

N'ot  present ... 
Generally  obtained... 

Headache  more  intense ; 

gets  worse  during  the 

course   of   the  illness. 

Hydrocephalic  cry. 
Frequent. 
Exceptional,    but    mny 

be  present. 

Wiclal's  rcactio)i    ... 

Usually  obtained  at 
the  end  of  ten  days 

Not  present. 

Rash 

Typhoid  spots  appear 
in       crops       from 
seventh     to    tenth 
day  aod  onwards 

Not  present. 

Squint 

Tubercle    in    other 

Not  present ... 
Absent          

Very  frequent. 
Suggestive,    though    of 

parts 

course  not  necessarily 
diagnostic. 

Temperature 

Fairly  regular  course 

More  irregular. 

Difficult  cases  can  only  be  diagnosed  by  a  careful 
review  of  all  the  symptoms  in  relation  to  one  another, 
for  nothing  is  more  frequent  than  for  exceptional  and 
deceptive  signs  to  appear  in  both  these  diseases. 

The  delirium  of  lohar  pneumonia,  especially  of  the 
apical  form,  is  sometimes  deceptive,  and  the  know- 
ledge that  the  pneumococcus  may  invade  the  meninges 
as  well  as  the  lungs  makes  the  diagnosis  still  more  un- 
certain in  doubtful  cases. 

When  the  case  appears  to  be  one  of  meningitis,  a 
careful  search  must  be  made  for  other  causes  before 
finally  concluding  that  it  is  tuberculous.     The  ears  must 


CEREBROSPINAL    FEVER  319 

be  examined  for  signs  of  suppuration,  and  any  possibility 
of  a  cerebral  abscess  must  be  carefully  considered.  It 
is  most  important  not  to  overlook  any  of  these  other 
causes  of  meningitis,  since  they  may  often  be  relieved 
by  surgical  treatment  when  the  operation  is  under- 
taken in  time. 

Lumbar  functure  may  be  an  aid  to  diagnosis.  In 
tuberculous  meningitis  the  fluid  is  generally  clear  or 
only  slightly  flaky.  Well-marked  lymphocytosis  is 
usually  present,  and  in  a  considerable  number  of  cases 
tubercle  bacilli  can  be  detected  by  staining  after  centri- 
fugalization. 

Treatment. — Frcm  the  foregoing  paragraphs  it 
may  be  gathered  that  no  drugs  have  any  specific  in- 
fluence on  the  disease.  Urotropine  may  be  given  for  its 
supposed  antiseptic  action  on  the  cerebro-sp)inal  fluid. 
Mercury  given  by  inunction  has  been  recommended,  but 
it  is  very  doubtful  if  it  is  of  any  value.  Potassium 
iodide  is  useless  in  tuberculous  meningitis,  but  should  be 
given  if  there  is  any  suspicion  as  to  the  affection  being 
syphilitic.  Relief  of  pressure  is  sometimes  obtained  by 
lumbar  puncture,  and  in  this  way  a  temporary  ameliora- 
tion of  the  symptoms  may  be  secured  ;  but  the  adhesions 
at  the  base  of  the  brain  usually  prevent  the  drainage 
being  sufficiently  free  to  give  any  permanent  relief. 

The  following  general  measures  should  be  adopted  : 
The  patient  should  be  kept  in  a  dark  room,  the  head 
shaved,  an  ice-bag  applied,  and  plenty  of  nourishment 
given.  Phenacetin,  antipyrin,  salicylates,  and  in  severe 
cases  opium,  may  be  given  for  the  relief  of  headache. 
The  bowels  should  be  kept  open. 

Signs  of  heart  failure  will  indicate  the  necessity 
for  digitalis  and  other  suitable  stimulants. 

II.  Epidemic  Cerebro-Spinal  Meningitis 
(Spotted  Fever) 
Etiology. — Epidemic  cerebro-spinal  meningitis  (cere- 
bro-spinal   fever   or   spotted   fever)   is   most   prevalent 
among  children  and  young  adults. 


320    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  micro-organism,  the  Diplococcus  intracellular  is 
of  Weichselbaum,  commonly  spoken  of  as  the  "  meningo- 
coccus," is  a  kidney-shaped  gram-negative  coccus,  and  is 
now  considered  to  be  identical  with  that  found  in  cases 
of  posterior  basic  meningitis,  described  later  in  this 
chapter. 

The  exact  conditions  which  cause  the  disease  to 
assume  an  epidemic  character  are  not  known,  but  atmo- 
spheric changes,  overcrowding,  and  chronic  catarrhal 
states  are  probably  factors  of  importance  in  modify- 
ing the  virulence  of  the  micro-organism.  Epidemics  are 
most  frequent  in  the  winter  and  spring  months. 

The  disease  is  infectious,  and  is  undoubtedly  spread 
to  a  great  extent  by  "  carriers,"  who  harbour  the  micro- 
organism in  the  throat,  and  so  give  the  disorder  to  others 
without  necessarily  suffering  from  it  themselves.  The 
organism  does  not  seem  to  possess  great  vitality  outside 
the  body,  but,  as  it  is  often  to  be  found  in  discharges 
from  the  nose  and  also  in  the  expectoration  of  patients, 
care  must  always  be  taken  to  prevent  the  disease  from 
spreading  by  these  means. 

The  micro-organism  is  not  very  easy  to  cultivate,  and 
grows  best  on  serum  agar.  During  life  it  can  be  obtained 
from  the  spinal  fluid  by  lumbar  puncture,  but  in  many 
instances  the  results  of  bacteriological  examination  of  the 
cerebro-spinal  fluid  are  negative,  especially  if  the  culti- 
vation is  not  made  quickly  after  the  fluid  is  withdrawn. 

Pathology. — The  meninges,  brain,  cord,  and  peri- 
pheral nerves  (both  cranial  and  spinal)  are  all  apt  to 
be  affected.  The  pia  and  arachnoid  membranes  are  the 
chief  seat  of  the  infection,  and  the  exudation,  which 
may  be  serous  or  purulent,  is  generally  most  intense  at 
the  base  of  the  brain,  although  it  often  extends  some 
distance  over  the  cerebral  hemispheres  and  the  lobes 
of  the  cerebellum.  In  the  most  acute  cases,  nothing 
more  than  intense  injection  of  the  pia  mater  may  be 
seen  with  the  naked  eye,  but  microscopical  examination 
generally  shows  that  a  considerable  amount  of  exuda- 
tion has  already  take  place. 


CEREBEO-SPINAL   EEVER  321 

In  the  chronic  cases  the  membranes  are  often  very 
much  thickened,  and  have  formed  adhesions  which 
may  interrupt  the  circulation  of  the  cerebro-spinal  fluid 
and  lead  to  hydrocephalus. 

Similar  changes  are  found  in  the  pia  mater  and  the 
arachnoid  of  the  cord,  more  especially  on  the  posterior 
Eurface. 

Patches  of  softening  and  haemorrhages  are  often 
present  in  both  the  cerebral  and  spinal  substances. 

The  inflammation  is  also  very  apt  to  extend  along 
the  cranial  and  spinal  nerves,  which  become  swollen  and 
softened  in  consequence.  It  is  stated  that  the  2nd, 
5th,  7th,  and  8th  are  the  most  liable  to  suffer,  and  that 
inflammatory  changes  of  an  advanced  character  can 
generally  be  found  in  the  Gasserian  ganglia. 

Symptoms. — The  meningeal  symptoms  are  often 
the  first  to  which  attention  is  directed,  though  in  many 
cases  the  history  shows  the  previous  presence  of  some 
naso-pharyngeal  catarrh,  and  in  other  instances  symp- 
toms of  general  septicaemia  precede  those  of  meningeal 
infection.  The  onset  of  meningitis  is  usually  very  acute, 
and  of  the  early  S3m[iptoms  the  most  prominent  are 
vomiting  and  fain,  the  latter  being  either  localized  to  the 
head  or  of  a  generalized  character. 

The  presence  of  meningitis  of  the  brain  and  cord 
is  soon  shown  by  palsies  of  the  cranial  nerves,  which 
cause  strabismus,  ptosis,  unequal  and  fixed  pupils,  facial 
paralysis,  and  deafness ;  by  rigidity  of  the  neck  and 
trunk  muscles,  with  retraction  of  the  head  and  opistho- 
tonos ;  by  o'ptic  neuritis,  convulsions,  hydrocefhalic  cry, 
Cheyne-Stokes  respiration,  delirium,  and  coma  ;  and  some- 
times by  hemiplegia  and  paraplegia.  The  mechanism  by 
which  these  symptoms  are  produced,  i.e.  by  irritation 
and  compression,  has  already  been  discussed  in  the 
section  on  Tuberculous  Meningitis,  to  which  the  reader 
is  referred. 

Inflammatory  conditions  of  the  eyes,  nose,  ears,  and 
lungs  are  often  present.  The  eye  symptoms  are  very 
important,  and  include  conjunctivitis,  iritis,  corneitis, 
V 


322    DISEASES   OF   THE  NERVOUS  SYSTEM 

and  sometimes  inflammation  of  all  the  structm:es  of 
the  eyeball,  which  may  result  in  permanent  blindness. 
Apart  from  these  changes,  a  temporary  loss  of  vision  may 
occur.  Disease  of  the  ears  is  a  complication  frequently 
met  with  ;  the  internal  ear  may  be  damaged  by  the 
extension  of  the  inflammation  along  the  auditory  nerve, 
to  which  reference  has  already  been  made ;  and  there 
is  often  acute  inflammation  of  the  middle  ear,  in  the 
discharge  from  which  the  specific  micro-organisms  may 
sometimes  be  found. 

Not  infrequently  there  is  a  discharge  from  the  nose, 
from  which  the  diplococci  may  also  be  isolated.  The 
bony  sinuses  of  the  skull,  too,  are  frequently  infected. 

The  organism  may  reach  the  lungs,  and  produce 
broncho-pneumonia. 

Small  haemorrhages  into  the  shin,  forming  petechial 
spots,  are  frequently  present ;  hence  the  name  "  spotted 
fever."  The  presence  of  herpes  has  also  been  frequently 
observed. 

The  jaulse  and  temperature  are  very  variable.  Swelling 
of  the  joints  sometimes  occurs. 

Clinical  course. — In  the  fulminating  cases,  death 
may  take  j)lace  within  twenty-four  hours.  The  ordinary 
acute  cases  generally  exhibit  all^the  main  symptoms  that 
have  been  detailed,  and  when  they  are  about  to  termi- 
nate fatally  the  coma  deepens  and  all  power  over  the 
sphincters  is  lost,  as  in  other  forms  of  meningitis. 

Sometimes  the  disease  runs  a  more  chronic  course, 
and  is  accompanied  by  great  emaciation  and  weakness, 
and — if  recovery  takes  place — by  impairment  of  the 
mental  faculties  or  hydrocephalus. 

Cases  in  which  there  are  intermission  and  remission 
of  symptoms  are  sometimes  observed. 

Diagnosis. — Differentiation  has  to  be  made  between 
this  disease  and  other  forms  of  meningitis,  such  as  those 
due  to  the  tubercle  bacillus  and  the  pneumococcus. 

To  decide  the  diagnosis,  lumbar  puncture  should 
be  performed,  and  the  fluid  thus  obtained  should  be 
examined   for  the  diplococci.     The   presence   of  these 


POSTERIOR    BASIC   MENINGITIS         323 

makes  tlie  diagnosis  conclusive;  but  too  much  im- 
portance must  not  be  attached  to  a  negative  result, 
for  the  organism  is  cultivated  with  difficulty,  and  may  be 
absent  during  the  remissions  and  also  in  the  later  stages. 

Prognosis. — The  death-rate  of  those  who  develop 
meningitis  is  very  high,  but  varies  in  difierent  epidemics 
according,  presumably,  to  the  virulence  of  the  organism. 
In  individual  cases  the  prognosis  must  be  based  upon 
the  intensity  of  the  general  infection  and  upon  the 
signs  denoting  the  degree  of  intracranial  pressure. 

Treatment. — ^AU  suspected  cases  should  be  isolated 
and  search  instituted  for  "carriers"  by  making  bac- 
teriological examinations  from  the  throats  of  contacts. 
Antimeningococcus  serum,  as  first  recommended  by 
Flexner,  has  proved  of  great  benefit,  and  the  mortality 
among  those  to  whom  the  serum  has  been  given  has  been 
greatly  reduced  as  compared  with  that  of  patients  not  so 
treated.  A  lumbar  puncture  should  first  be  performed, 
after  which  the  serum,  adjusted  in  amount  to  the 
quantity  of  cerebro-spinal  fluid  withdrawn,  should  be 
slowly  introduced,  preferably  by  the  gravity  method. 

An  attempt  to  relieve  the  intracranial  pressure 
may  be  made  by  performing  lumbar  puncture  at  short 
intervals,  and  in  some  cases  this  procedure  appears  to 
assist  materially  in  tiding  the  patient  over  the  critical 
period  of  the  disease. 

III.  Posterior  Basic  Meningitis 
Etiology. — In  this  form  of  meningitis  the  inflam- 
mation is  mainly  confined  to  the  posterior  part  of  the 
base  of  the  brain.  The  micro-organism,  first  noted  in 
connection  with  this  disease  by  Still,  has  proved  to 
be  identical  with  the  meningococcus  of  epidemic  cerebro- 
spinal meningitis,  of  which  posterior  basic  meningitis 
must  be  considered  to  be  a  sporadic  form. 

The  organism  probably  reaches  the  membranes  of 
the  brain  by  way  of  the  Eustachian  tube  and  middle 
ear,  and  any  debilitating  process,  such  as  rickets,  injury, 
or  catarrh,  renders  the  child  more  liable  to  infection. 


324    DISEASES   OF   THE  NERVOUS   SYSTEM 

Children  of  both  sexes  are  attacked,  and  the  majority 
of  cases  occur  during  infancy. 

Pathology. — The  exudate  is  found  over  the  posterior 
part  of  the  base  of  the  brain,  and,  if  it  has  existed  long 
enough,  there  may  be  occlusion  of  foramina  in  connection 
with  the  4th  ventricle  and  interruption  of  the  flow 
of  cerebro-spinal  fluid ;  the  latter  then  collects  in  the 
lateral  ventricles  and  produces  hydrocephalus.  Some- 
times the  inflammation  extends  some  way  down  the 
cord  or  on  to  the  vertex  of  the  brain. 

Symptoms. — The  onset  is  usually  acute,  but  the 
symptoms  may  be  indefinite  for  a  time.  Vomiting 
occurs  early,  and  is  generally  accompanied  or  quickly 
followed  by  retraction  of  the  head,  which  is  one  of  the 
most  prominent  features  of  the  disease  (Plate  15). 

More  widely  spread  tonic  spasms  cause  general 
rigidity,  or  even  opisthotonos ;  convulsions  may  also 
occur.  The  rise  of  intracranial  pressure  is  shown  by  the 
tense  fontanelles,  and  it  is  probably  the  cause  of  the 
temporary  blindness  which  is  often  present,  since  optic 
neuritis  is  seldom  met  with.  The  condition  of  the 
pupils  varies ;  in  some  cases  they  are  contracted,  in 
others  dilated.  Owing  to  retraction  of  the  upper  lids, 
the  eyes  often  appear  unduly  prominent ;  nystagmus 
may  sometimes  be  observed.  Strabismus  often  occurs, 
though  not  with  such  frequency  as  in  tuberculous 
meningitis. 

Alterations  resembling  those  of  tuberculous  meningitis 
may  take  place  in  the  pulse  and  respiration. 

The  deep  reflexes  vary  in  their  reactions,  and  arc 
sometimes  increased,   sometimes  absent. 

In  the  later  stages  the  mental  powers  may  be  im- 
paired by  hydrocephalus. 

Diagnosis. — ^At  the  onset  it  is  often  difficult  or  even 
impossible  for  a  time  to  decide  whether  meningitis  or 
some  other  acute  febrile  condition,  e.g.  pneumonia,  is 
present.  After  a  few  days  the  ref erence  .^of  symptoms 
to  the  head,  together  with  the  absence  of  signs  of  a 
generalized  infection,  makes  the  locality  of  the  disease 


X 


;  > 


PLATE  15.— Case  of  posterioi-  basic  meningitis  showing  extreme 
retraction  of  the  head. 

{From  a  sketch  by  Dr.  Penrose  Watkin  Williams.) 


SEROUS   MENINC4ITIS  325 

more  apparent,  and  it  is  then  necessary  to  exclude 
the  presence  of  sinus  thrombosis,  otitis  media,  and 
mastoid  disease  before  arriving  at  the  diagnosis  of 
meningitis.  When  the  diagnosis  of  meningitis  has 
been  made,  it  is  still  necessary  to  attempt  to  decide 
the  nature  of  the  infection,  and  the  chief  difficulty 
lies  in  differentiating  the  posterior  basic  from  the  tuber- 
culous form. 

Eeference  to  the  list  of  symptoms  shows  that  in 
posterior  basic  meningitis  the  rigidity  and  retraction 
of  the  head  are  the  most  prominent  symptoms,  while 
optic  neuritis,  palsies  of  cranial  nerves,  and  retraction 
of  the  abdomen  are  less  likely  to  be  present.  The 
detection  of  tubercles  in  the  choroid  will,  of  course, 
decide  the  question,  as  also  may  a  bacteriological  ex- 
amination of  the  cerebro-spinal  fluid.  A  blood-count 
may  also  be  of  value,  for  in  posterior  basic  meningitis 
leucocytosis  is  usually  greater  than  that  which  occurs 
in  tuberculous  meningitis. 

Prognosis. — The  prognosis  of  posterior  basic  menin- 
gitis is  not  altogether  unfavourable.  A  considerable 
number  of  patients  get  well,  though  often  with  mental 
impairment,  the  result  chiefly  of  the  attendant  hydro- 
cephalus. The  duration  of  the  illness  varies,  but  it 
often  lasts  several  weeks,  and  thus  may  be  longer  than 
in  the  tuberculous  form. 

Treatment. — Mercury  may  be  given  on  account 
of  its  general  tendency  to  influence  inflammatory  pro- 
cesses, and  it  is  well  borne  by  children.  Iodides  have 
also  been  recommended.  Operations  with  a  view  to 
draining  off  the  excess  of  fluid  and  relieving  the  pressure 
have  not  met  with  any  constant  success,  but  cases 
have  been  recorded  in  which  repeated  lumbar  punctures 
have  been  followed  by  beneficial  results. 

IV.  Serous  Meningitis 
In  this  variety,  which  is  also  known  as  ''  wet  brain," 
the  exudate  is  serous    in   character   and  often  more 
oedematous    than    inflammatory.     The    condition    has 


326    DISEASES   OF  THE  NERVOUS  SYSTEM 

been  more  particularly  described  in  association  with 
chronic  alcoholism,  but  it  may  be  found  in  other  cir- 
cumstances, and  more  recently  probable  cases  have 
occasionally  appeared  to  occur  in  association  with 
attacks  of  lethargic  encejDhalitis  (see  p.  346). 

The  symptoms  may  resemble  those  of  other  forms 
of  meningitis,  but  are  usually  less  intense.  The  general 
signs  of  increased  intracranial  pressure  are  shown  by 
headache,  optic  neuritis,  and  vomiting,  with  which  may 
be  associated  various  signs  of  cranial  nerve  disturbance. 
When,  as  sometimes  happens,  the  temperature  is  sub- 
normal the  condition  may  closely  resemble  cerebral 
tumour. 

With  rest  the  symj^toms  may  subside,  and  relief 
is  also  sometimes  obtained  by  lumbar  puncture. 
If  no  improvement  takes  place,  or  if  the  vision  is  in 
danger  of  permanent  deterioration,  the  question  of 
performing  an  operation  for  cerebral  decompression 
should  be  considered. 

V.  Suppurative  Meningitis 

Etiology. — The  invasion  of  the  meninges  by  pyo- 
genic organisms  may  take  place  from  extension  of  local 
suppuration,  or  it  may  be  a  part  of  the  terminal  stage 
of  a  general  infection.  The  local  conditions  which 
most  frequently  give  rise  to  meningitis  are  suppura- 
tive otitis  media,  mastoid  disease,  and  cerebral  abscess, 
all  of  which  are  frequently  closely  associated  with  one 
another. 

The  symptoms  of  suppurative  meningitis  do  not 
materially  difier  from  those  described  under  the  heading 
of  Tuberculous  Meningitis,  since  they  also  depend  upon 
the  irritation  and  pressure  of  exudation.  The  general 
tendency,  however,  is  for  the  suppurative  forms  to  spread 
over  the  convexity  of  the  brain  rather  than  to  remain 
confined  to  the  base  ;  hence  the  functions  of  the  cranial 
nerves  are  apt  to  be  less  extensively  deranged  than  in 
the  tuberculous  form. 

Suppurative   meningitis  is   accompanied   by   severe 


EXTERNAL   PACHYMENINGITIS  327 

general   symptoms,   and   when   diffused   always   causes 
death. 

The  treatment  consists  in  relieving  the  symptoms 
on  the  lines  already  mentioned.  When  the  meningitis 
is  once  established  the  opportunity  for  surgical  inter- 
ference has  passed,  and  it  is,  therefore,  most  important 
to  deal  promptly  with  any  local  suppurative  process 
about  the  skull  before  any  extension  to  the  membranes 
has  taken  place. 

VI.  Pachymeningitis  Externa 

In  some  cases  where  there  is  direct  extension  from 
the  bones  of  the  skull  the  suppuration  is  for  a  time,  at 
any  rate,  confined  to  the  outer  surface  of  the  dura 
(pachymeningitis  externa),  and  if  a  collection  of  pus 
be  localized,  incision  and  drainage  may  be  attended  with 
success.  Left  to  itself,  the  natural  tendency  is  for  the 
pua  to  infect  the  inner  membranes. 

VII.  Injuries  to  the  Meninges 

Among  the  common  causes  of  injuries  to  the  meninges 
are  damage  to  the  skull  during  childbirth  and,  in  later  life, 
blows  on  the  head.  One  of  the  most  serious  injuries  is  a 
blow  on  the  head  causing  haemorrhage  from  the  middle 
meningeal  artery  or  one  of  its  branches.  The  immediate 
effects  of  a  blow  of  this  kind  may  not  appear  to  be 
serious,  but  after  a  brief  interval  of  time  the  results  of 
the  haemorrhage  make  themselves  felt.  The  patient 
becomes  dazed  and  soon  loses  consciousness ;  signs  of 
progressive  hemiplegia,  with  or  without  convulsions, 
develop  ;  the  loss  of  consciousness  deepens,  and  the 
patient  rapidly  becomes  comatose  and  dies  unless  the 
skull  can  be  opened  and  the  bleeding  quickly  arrested. 

Experience  of  war  injuries  has  shown  that  damage 
to  the  membranes  and  to  the  cortex  beneath  may  be 
produced  by  high  velocity  missiles  merely  grazing  the 
bones.  Wilfred  Trotter  *  has  investigated  this  condition 
and  concludes  that  "  the  resilience  of  the  skull  is  such 
*  Brain,  vol.  xlii.,  pt.  iv„  1919, 


32B    DISEASES  OF  THE  NERVOUS  SYSTEM 

that  whenever  a  missile  travelling  at  high  velocity 
touches  the  bone,  the  latter  is  driven  in  upon  the  brain 
with  force  enough  to  inflict  a  considerable  bruise,"  and 
that  in  such  circumstances  a  contusion  of  the  brain  is 
likely  to  remain  longer  than  it  would  in  other  organs, 
owing  to  the  unyielding  character  of  the  cranium,  which 
prevents  the  compensatory  swelling  by  which  the  circu- 
lation is  maintained  through  other  bruised  organs.  The 
principal  symj)toms  in  such  a  /^ase  are  headaches,  made 
worse  by  exertion,  especially  stooping,  and  giddiness, 
which  symptoms,  Trotter  finds,  can  be  greatly  relieved 
or  cured  by  free  removal  of  bone  and  incision  of  the 
dura  mater  at  the  seat  of  injury,  the  opening  being 
closed  again  some  months  later.  On  operation  the 
arachnoid  is  found  to  be  oj^aque  and  distended  by  a  local 
accumulation  of  fluid  beneath  it,  and  the  surface  of  the 
brain  is  discoloured  from  old  extra vasated  blood. 


CHAPTER   XXXV 

CEREBRAL  PALSIES  OF  CHILDREN  ;     AMAUROTIC 
FAMILY  IDIOCY ;  HYDROCEPHALUS 

CEREBRAL  PALSIES   OF   CHILDREN 

The  paralyses  of  cerebral  origin  which  occur  in  child- 
hood may  be  grouped,  according  to  their  causation,  as 
follows  : 

(a)  Before  hirih :  e.g. 

(1)  Primary  arrest  of  development. 

(2)  Changes  depending  on  maternal  syphilis 

or  some  other  toxic  condition. 

(6)  During  birth  :  e.g. 

(1)  Hsemorrhages  into  the  meninges. 

(2)  Direct  injury  of  cerebral  cortex, 
(c)  After  birth  :  e.g. 

(1)  Acute  encephalitis. 

(2)  Thrombosis, 

(3)  Meningeal  hsemorrhage. 

(4)  Meningitis. 

(5)  Cerebral  tumours. 

(6)  Nerve  degeneration. 

Pathology. — ^As  might  be  expected  from  the  variety 
of  the  causal  lesions,  the  brains  differ  very  markedly 
from  one  another  in  their  appearance. 

In  some,  an  abortive  development  of  the  motor 
areas  is  the  main  feature — a  condition  known  as  cerebral 
agenesis.  In  others,  the  brain  is  small,  and  exhibits  a 
generalized  sclerosis  ;  while  in  others  again,  cystic  con- 
ditions, known  as  porencephaly,  are  found. 

329 


330    DISEASES   OF   THE  NERVOUS  SYSTEM 

Thrombosis  of  vessels  and  softening  of  brain  tissue 
occur  from  encephalitis,  and  may  give  rise  later  on  to  a 
scar,  over  which  the  membranes  may  be  thickened  and 
adherent. 

The  paralysis  usually  takes  the  form  of  a  diplegia  or 
of  a  hemiplegia. 

Cerebkal  Diplegia 

Cerebral  diplegia  is  characterized  by  spasticity  of 
both  sides  of  the  body,  and  by  a  variable  degree  of 
weakness.  Arrested  development  of  the  motor  areas 
during  intra-uterine  life  appears  to  be  the  most  common 
cause.  The  nerve  elements  which  should  make  up  the 
motor  areas  of  the  brain  either  fail  to  develop,  or  degener- 
ate during  the  period  of  their  development,  poisoned  by 
syphilis  or  some  other  toxin.  In  some  cases  they  appear 
to  atrophy  from  sheer  want  of  vitality,  and  so  form  a 
connecting  link  with  those  in  which  the  degeneration 
occurs  in  later  childhood — e.g.  in  Friedreich's  disease  and 
some  of  the  myopathies — a  connection  which  is  further 
emphasized  by  the  occasional  tendency  of  cerebral 
diplegia  to  appear  in  different  members  of  the  same 
family. 

All  gradations  exist  between  complete  absence  of  the 
motor  areas  and  slight  impairment  of  their  functions, 
with  corresponding  clinical  variations.  When  the  damage 
to  the  brain  occurs  during  the  birth  of  the  child,  it  is 
most  probably  due  to  a  hsBmorrhage  into  the  meninges 
whereby  the  motor  areas  of  the  legs  are  more  or  less 
symmetrically  injured,  the  lesion  sometimes  extending 
as  far  over  the  hemispheres  as  the  areas  corresponding 
to  the  movements  of  the  upper  extremities. 

After  birth  the  injuries  are  far  more  likely  to  be  uni- 
lateral, and  to  cause  hemiplegia  rather  than  diplegia. 

It  very  frequently  happens  that  the  exact  period  at 
which  the  damage  occurred  cannot  be  ascertained  with 
any  degree  of  certainty  from  the  history  of  the  case. 

Symptoms. — The  main  symptoms  of  cerebral  di- 
plegia are  rigidity,  weakness,  involuntary  movements,  and 
mental  infirmity,  but  their  variability  in  degree  is  very 


CEEEBKAL   DIPLEGIA 


331 


great,  so  that  cases  differ  very  considerably  from  one 
another. 

In  a  typical  case  of  cerebral  diplegia  it  is  noticed 
that  the  child's  limbs  are  stiff,  and  that  the  movements 
are  not  so  free  as  they  should  be. 

On  examination,  there  is  evidence  that  the  child  has 
the  signs  of  degeneration  of  the  motor  tracts,  as  shown 
by  weakness,  rigidity,  and  increased  knee-jerks.     The 
behaviour  of  the  plantar  reflex 
is   not   of   much    assistance   in 
young  infants,  since  in  them  it 
is  normally  of  the  extensor  type, 
but  in  older  children  it  possesses 
the  usual  ^gnificance. 

As  a  rule,  the  rigidity  is 
rather  out  of  proportion  to  the 
weakness,  and,  on  the  patient 
attempting  to  walk,  the  well- 
known  "  scissors-legged  "  atti- 
tude is  assumed,  owing  to  pre- 
dominant spasm  of  the  adductors 
of  the  thigh  (Fig.  86).  The  arms 
share  in  the  rigidity,  though 
oftentimes  to  a  less  degree.  The 
face  may  also  be  affected,  and 
occasionally  there  is  coincident 
atrophy  of  the  optic  nerves. 

The  weakness,  always  present 
in  some  degree,  is  often  rather 
masked  by  the  rigidity  which  makes  voluntary  move- 
ments far  more  difficult  than  they  would  otherwise  be. 

Involuntary  movements  occur  in  a  considerable 
number  of  cases,  and  often  attack  the  hand  and  arm 
(less  commonly  the  leg  and  face),  taking  the  form  known 
as  athetosis,  the  chief  characters  of  which  are  slow, 
more  or  less  rhythmical  "  squirming,"  involuntary  move- 
ments of  the  fingers  and  thumbs,  in  which  hyper- 
extension  is  a  prominent  feature.  Sometimes  the  whole 
arm  takes  part   in  the  movements,  which  may  be  so 


Fig.  83.— "Scissors-legged  " 
progression  in  a  case 
of  cerebral  diplegia. 


332    DISEASES   OF  THE  NERVOUS   SYSTEM 

violent  and  uncontrollable  as  to  necessitate  strapping 
the  limb  to  the  side.  Other  movements  may  take  the 
form  of  tremors,  or  may  be  of  a  more  irregular  chorei- 
form nature  (Plate  16). 

All  degrees  of  mental  impairment  are  met  with, 
from  "  backwardness  "  to  complete  idiocy.  Articulation 
also  is  often  impaired. 

Epileptic  fits  are  frequently  associated  with  cerebral 
diplegia. 

The  less  severe  cases  of  diplegia,  in  which  the  symp- 
toms are  confined  to  the  legs,  and  consist  of  some  rigidity 
combined  with  weakness  of  only  moderate  degree,  are 
often  classed  together  in  a  group  under  the  heading 
of  Little's  disease,  after  the  name  of  the  surgeon  who 
first  described  them.  An  "atonic"  form,  in  which 
myatonia  is  a  prominent  symptom,  has  also  been 
described. 

Diagnosis. — When  the  symptoms  are  pronounced, 
there  is  but  little  difficulty  in  recognizing  their 
nature. 

The  limbs  of  children  suffering  from  severe  rickets 
or  scurvy  rickets  sometimes  appear  to  be  paralysed, 
but  the  absence  of  rigidity,  with  the  enlargement  of  the 
ends  of  the  long  bones,  and  other  signs  of  rickets,  shows 
that  the  weakness  is  not  of  nervous  origin. 

In  older  children,  with  symptoms  of  a  moderate 
degree,  disseminated  sclerosis  and  Friedreich's  disease 
may  require  consideration.  Disseminated  sclerosis  is 
very  rare  in  childhood,  and  possibly  some  of  the  cases 
recorded  have  been,  in  reality,  cerebral  diplegias. 

Variability  of  symptoms  and  nystagmus  may  help 
to  distinguish  difficult  cases,  and,  of  course,  the  history, 
when  obtainable,  is  a  most  reliable  aid. 

It  is  important  to  differentiate  the  two  diseases 
carefully,  since  the  outlook  for  those  suffering  from 
disseminated  sclerosis  is  not  good,  whereas  the  slighter 
forms  of  cerebral  diplegia  often  tend  to  ira23rove. 

Friedreich's  disease  comes  on  later  in  life,  and  the 
ataxy,    loss   of   deep    reflexes,    nystagmus,    and    other 


PLATE  16.— Instantaneous  photograph  showing  bilateral 
athetosis  in  a  case  of  cerebral  diplegia 
{By  courtesy  of  Dr.  George  Ogilvie.) 


INFANTILE   HEMIPLEGIA  333 

characteristic  symptoms  will  generally  enable  it  to  be 
distinguislied. 

Prognosis  and  treatment. — Extraordinary  im- 
provements can  sometimes  be  effected  in  the  less  severe 
cases  by  patiently  persevering  with  mental  and  physical 
"education ;  for  the  latter  carefully  regulated  gymnastics 
should  be  prescribed.  Pronounced  mental  impairment 
and  frequent  fits  are  of  evil  significance,  and  the  onset 
of  athetosis  usually  indicates  that  the  chance  of  further 
improvement  in  the  use  of  the  limbs  is  but  slight. 

In  cases  where  the  difficulty  in  using  the  limbs  is 
due  to  excessive  rigidity  rather  than  loss  of  power, 
improvement  has  sometimes  been  obtained  by  Foster's 
operation  of  dividing  the  posterior  nerve  roots.  By 
this  procedure  the  rigidity  may  be  materially  diminished, 
but  care  must  be  taken  to  divide  only  a  certain  number 
of  nerve  roots  to  each  limb,  for  were  all  divided  the  part 
would  be  rendered  useless  through  the  total  anaesthesia 
and  loss  of  muscle  tone  that  would  naturally  result. 

In  other  instances  the  power  of  movement  may 
be  improved  by  stretching  or  dividing  the  spastic 
muscles. 

All  cases  for  operation  should  be  carefully  considered 
from  the  mental  as  well  as  the  physical  aspect  of  the 
patient,  for  improvement  ultimately  depends  largely 
upon  the  success  with  which  postoperative  treatment 
by  systematic  exercises  and  re-education  of  movements 
can  be  carried  out. 

Infantile  Hemiplegia 

The  hemiplegias  of  childhood  are,  for  the  most  part, 
due  to  injuries  received  at  the  time  of  birth  or  to  post- 
natal inflammatory  and  vascular  lesions. 

During  prolonged  and  difficult  labours,  haemorrhage 
or  some  other  injury  may  occur  by  which  the  functions 
of  the  cortical  motor  areas  are  impaired. 

After  birth,  certain  blood  states,  especially  those 
brought  about  by  specific  fevers,  are  conducive  to 
thrombosis  in  the  cerebral  vessels,  or  the  direct  invasion 


334    DISEASES  OF  THE  NERVOUS  SYSTEM 

of  the  brain  tissue  by  micro-organisms  may  give  rise 
to  an  acute  encephalitis.  The  most  important  of  the 
latter  are  the  forms  known  as  acute  polioencephalitis,  in 
which  the  toxin  ajDpears  to  be  identical  with  that  of  acute 
poliomyelitis  {see  p.  189),  and  encephalitis  lethargica 
{see  p.  341). 

Symptoms. — Of  the  cases  due  to  polioencephalitis, 
most  occur  in  children  between  the  ages  of  3  and  6,  and 
the  onset  is  generally  marked  by  fever,  malaise,  and  con- 
vulsions, with  more  or  less  disturbance  of  consciousness. 

Between  the  convulsions  that  attend  the  onset  of 
the  disease,  or  more  frequently  after  the  lapse  of  one 
to  several  days,  unilateral  weakness  is  observed,  generally 
most  pronounced  in  the  upper  extremity  ;  and  in  some 
cases  the  motor  nuclei  of  cranial  nerves  are  affected  like 
those  of  the  cord  in  anterior  poliomyelitis. 

Sometimes  the  power  gradually  returns  to  the 
affected  parts,  but  in  the  majority  of  cases  a  considerable 
amount  of  paralysis  persists.  The  later  symptoms  are, 
in  the  main,  those  already  described  in  the  diplegias, 
viz.  fits,  mental  deterioration,  and  perhaps  athetosis. 

When  the  lesion  is  in  the  cortex  of  the  left  hemisphere 
there  is  the  possibility  of  aphasia,  but  the  right  side  will 
probably  take  up  the  function  of  speech  if  the  subject 
is  a  young  child. 

Encephalitis  lethargica  is  less  common  in  childhood, 
but  the  lasting  effects  of  any  local  damage  to  the  brain 
from  this  cause  will  be  similar  to  those  of  polioencepha- 
litis described  above. 

Diagnosis. — The  convulsions  and  distribution  of 
the  paralysis  indicate  a  cerebral,  as  opposed  to  a  spinal, 
lesion,  while  fever,  and  drowsiness  or  complete  loss  of 
sensibility,  are  the  usual  accompaniments  of  cases  of 
acute  polioencephalitis.  The  presence  of  a  valvular  lesion 
of  the  heart  will  suggest  the  possibility  of  embolism. 

When  a  long-standing  case  is  seen  for  the  first  time, 
its  cerebral  nature  will  be  evident  from  the  spasticity, 
increased  reflexes,  and  absence  of  electrical  changes,  in 
contrast  to  the  flaccidity,  the  loss  of  reflexes,  and  the 


CEREBELLAR  PALSIES  335 

reaction  of  degeneration  found  in  the  paralysis  following 
acute  anterior  poliomyelitis  of  the  cord.  The  atrophy 
is  much  more  evident  in  cases  following  the  latter  lesion, 
although  the  limbs  of  the  infantile  hemiplegic  also  look 
wasted  owing  to  arrest  of  their  development,  and  club- 
foot is  a  common  occurrence  in  both  diseases. 

Treatment. — During  the  febrile  period  of  cases  of 
acute  encephalitis  the  child  should  be  kept  quiet  in  bed 
and  the  room  darkened.  A  mixture  containing  bromides 
and  a  purge  of  calomel  or  grey  powder  should  be  given, 
and  the  diet  should  be  as  Ught  as  possible.  When  the 
signs  of  cortical  irritation  have  quite  disappeared,  the 
most  careful  and  persistent  attention  must  be  given 
to  the  paralysed  limbs.  Passive  and,  where  possible, 
active  movements  must  be  practised  regularly,  and  every 
possible  efiort  should  be  made  to  prevent  the  onset  of 
contractures. 

The  ultimate  results  in  many  instances  are  surpris- 
ingly good,  but  no  accurate  prognosis  can  be  given  for 
any  individual  case,  since  the  onset  of  fits,  athetosis,  or 
permanent  mental  disability  may  mar  the  most  promising 
outlook. 

When  fits  occur  they  must  be  treated  with  bromide,  as 
in  ordinary  epilepsy. 

The  possibilities  of  treatment  by  surgical  methods  are 
similar  to  those  discussed  in  the  preceding  section 
dealing  with  diplegia. 

Cerebellar  Palsies  of  Childhood 
The  causes  of  the  cerebellar  lesions  of  childhood 
are  very  similar  to  those  producing  the  cerebral  palsies, 
and  may,  in  like  fashion,  be  divided  into  those  occurring — 
{a)  Before  birth. 

(b)  During  birth. 

(c)  After  birth. 

The  main  symptom  in  cases  of  cerebellar  disease  in 
children  is  ataxia,  and  F.  E.  Batten  *  has  shown  that 
cases   of   ataxia  in  childhood  in  relation  to  cerebellar 
*  Brain^  1905,  Pts.  cxi.,  cxii. 


336    DISEASES   OF  THE   NERVOUS  SYSTEM 

disease  may,  from  a  clinical  point  of  view,  be  classified 
as  follows  : 

(1)  Congenital  cerebellar  ataxia;    in  which  the 

ataxia  has  been  noticed  from  earliest  life. 

(2)  Acute  cerebellar  ataxia  ;   due  to  encephalitis 

of  the  cerebellum,  in  which  the  ataxia 
has  developed  during  the  course  of  or 
after  some  acute  illness  in  a  child  who 
was  formerly  healthy. 

(3)  Progressive   cerebellar  ataxia ;  in  which   the 

ataxia  has  gradually  developed  in  a  child 
who  has  previously  been  healthy  and  of 
normal  development. 

Tlie  value  of  this  classification  is  enhanced  by  F.  E. 
Batten's  observations  on  the  prognosis,  which  led 
him  to  conclude  that  the  cases  which  come  under  the 
heading  of  congenital  ataxia,  and  those  also  which  are 
due  to  polioencephalitis,  show  a  tendency  to  improve 
gradually,  while  in  those  belonging  to  the  group  of  pro- 
gressive ataxia  the  outlook  is  bad. 

AMAUROTIC  FAMILY  IDIOCY 
(WAREN  TAY'S  DISEASE;   SACHS'  DISEASE) 

This  disease,  which  Sachs  has  termed  amaurotic 
family  idiocy,  is  characterized  by  progressive  degener- 
ation of  nerve  cells  throughout  the  whole  of  the 
nervous  system. 

Etiology. — There  is  a  great  tendency  for  the  disease 
to  appear  in  several  members  of  a  family,  and  hitherto 
all  the  recorded  cases  about  the  nature  of  which  there 
is  no  doubt  have  been  children  of  Jewish  parents.  The 
children,  apparently  healthy  when  first  born,  develop 
symptoms  within  the  first  few  months. 

Different  views  are  held  concerning  the  probability 
of  a  congenital  or  an  acquired  origin  of  the  disease  : 
the  family  characteristics  certainly  point  to  a  congenital 
origin,  and  the  idea  that  abnormalities  of  the  mother's 
milk  may  be  a  cause  appears  to  have  been  disproved  by 


PLATE  17— The  fundus  oculi,  showing  the  characteristic  appearance  in 
amaurotic  family  idiocy. 

(By  courtesy  of  Mr.  ILbert  Hancock.) 


AMAUROTIC   FAMILY    IDIOCY  337 

Sachs,  who  caused  several  of  his  patients  to  be  reared  by 
wet-nurses.  Gordon  Holmes  and  Poynton,  after  care- 
ful consideration  of  cases  which  they  have  examined, 
think  that  the  disease  is  due  neither  to  arrested  de- 
velopment nor  to  bacterial  toxins,  but  depends  upon 
"  some  inherent  biochemical  property  of  the  proto- 
plasm of  the  cells  "  which  brings  about  the  degenerative 
processes. 

Pathology. — There  is  a  widespread  degeneration 
of  the  cellular  elements  of  the  brain  cortex,  with  various 
degrees  of  sclerosis.  Changes  have  also  been  noted 
in  the  basal  ganglia  and  grey  matter  of  the  cord.  Mott  * 
concludes  that  the  essential  feature  is  failure  in  the 
elaboration  of  the  nucleo-proteins  of  the  neurones,  as 
evidenced  histologically  by  the  disappearance  of  the 
Nissl  granules. 

Symptoms. — The  symptoms  usually  appear  in 
the  first  few  months  of  life — mostly  between  the  third 
and  sixth.  The  child  becomes  weak  and  wasted,  and 
there  is  soon  an  obvious  paralysis,  which  affects  most 
of  the  body,  and  which  may  at  first  be  of  flaccid, 
and  later  on  of  spastic  type.  At  the  same  time  the 
power  of  vision  fails  and  the  mental  faculties  rapidly 
deteriorate. 

The  wasting  and  weakness  progress,  and  death 
usually  takes  place  in  a  few  months. 

The  condition  of  the  reflexes  varies  with  the  spastic 
and  flaccid  nature  of  the  paralysis. 

The  ocular  symptoms,  first  described  by  Waren  Tay, 
are  very  characteristic  of  the  disease,  for,  in  addition 
to  optic  atrophy,  there  is  in  the  region  of  the  macula 
lutea  a  whitish  patch,  in  the  middle  of  which  is  a  round 
spot  of  cherry-red  colour.  The  appearance  of  this  is 
similar  to  that  occurring  after  embolism  of  the  central 
artery  of  the  retina  (Plate  17). 

Prognosis  and  treatment. — -Up  to  the  present, 
no  methods  of  retarding  the  coui'se  of  the  degeneration 
have  been  discovered. 

*  Arch,  of  Neurology,  vol.  iii.,  1907. 
W 


338    DISEASES  OF  THE  NERVOUS  SYSTEM 

HYDROCEPHALUS 

Hydrocephalus  is  characterized  by  the  accumulation 
of  serous  exudation  within  the  skull. 

Internal  hydrocephalus  is  the  form  usually  met  with, 
and  may  be  the  result  of  many  different  underlying 
pathological  conditions. 

External  hydrocephalus  is  the  term  applied  to  the 
collection  of  fluid  between  the  dura  mater  and  the  brain, 


Fig.  87.— Hydrocephalic  skull.    (Museum,  Middlesex  Hospital ; 
Sir  John  Bland-Sutton.) 

but  the  symptoms  to  which  it  gives  rise  are  usually 
masked  by  the  more  important  changes  on  which  it 
depends, 

Acute  hydrocephalus  occurs  in  association  with 
various  forms  of  meningitis. 

Chronic  hydrocephalus  may  be  due  to  some  congenital 
condition,  or  it  may  arise  after  birth  as  the  result  of 


HYDROCEPHALUS 


339 


meningeal  inflammation  or  tumours.  In  many  cases 
the  chief  factor  seems  to  be  obliteration  of  the  foramen 
of  Magendie  and  other  passages  through  which  the  fluid 
normally  circulates,  added  to  which  there  may  be  an 
excessive  secretion  of  the  fluid. 


Fig.  88.— Sagittal  section  of  a  hydrocephalic  skull  from  a  child, 

with  the  brain  in  situ.    The  head  of  the  arrow  is  in  the  4th 

ventricle,  and  its  feathers  are  in  the  3rd.    The  infundibulum 

is   widely   dilated.     (Mitseum,   Middlesex   Hospital;     Sir  John 

Bland-Sutton.) 

The  distension  of  the  ventricles  usually  causes  altera- 
tions in  the  brain,  leading  to  mental  impairment,  but  in 
some  instances  the  intellect  has  remained  good.  Various 
degrees  of  diplegia  may  also  be  present. 


340  DISEASES   OF    THE    NERVOUS    SYSTEM 

In  the  hydrocephalic  skull  (Figs.  87,  88)  the  bones 
are  separated  and  the  fontanelles  distended.  The  frontal 
and  parietal  bones  bulge  outwards  and  overhang  the 
eyes,  the  eyeballs  being  somewhat  depressed  by  the 
pressure  of  the  orbital  plates.  The  bones  are  sometimes 
softened,  and  Wormian  bones  occupy  the  parietal  sutures. 
Distinction  must  be  made  between  the  hydrocephalic 
head  and  the  square,  flatter-topped  head  of  rickets. 

Prognosis. — ^In  most  cases  of  hydrocephalus  the 
intellect  is  impaired,  and  the  child  usually  dies  young. 

Treatment. — The  most  rational  method  of  treat- 
ment is  that  of  draining  the  fluid  away,  but  hitherto 
the  various  means  adopted  have  not  met  with  much 
success. 


CHAPTER   XXXVl 

ACUTE  ENCEPHALITIS  ;    CEREBRAL  ABSCESS 

ACUTE  ENCEPHALITIS 

Some  generalized  inflammation  of  the  surface  of  the 
brain  probably  always  occurs  in  association  with  menin- 
gitis, and  to  a  more  limited  extent  after  injuries  such  as 
fractures  and  punctured  wounds  of  the  skull.  It  may 
also  be  associated  with  areas  of  softening  due  to  embolism 
or  thrombosis. 

A  more  localized  form  of  inflammation,  to  which 
the  name  polioencephalitis  has  been  given,  corresponds  to 
acute  anterior  poliomyelitis  of  the  cord,  and  is  charac- 
terized by  an  acute  inflammation  of  groups  of  cells  in 
the  cerebral  cortex,  or  the  cerebellum,  or  the  nuclei 
of  the  cranial  nerves.  The  resulting  palsies,  which  are 
mainly  of  hemiplegic  or  cranial  nerve  distribution,  are 
alluded  to  under  the  head  of  Cerebral  Palsies  of  Children 
(p.  329). 

More  recently  the  disease  known  as  encephalitis 
lethargica,  also  frequently  associated  with  localized  in- 
flammations of  the  brain,  has  been  recognized. 

Encephalitis  Lethaegica  (Epidemic  Encephalitis) 

Attention  was  directed  to  the  occurrence  of  epidemic 
encephalitis  in  this  country  in  1918  by  Wilfred  Harris, 
who  described  a  series  of  cases  characterized  by  ocular 
palsies  under  the  title  of  Acute  Infective  Ophthalmo- 
plegia. About  the  same  time  A.  J.  Hall  also  noticed  the 
occurrence  of  a  series  of  similar  cases  in  the  neighbour- 
hood of  Sheffield.  It  was  thought  at  first  that  the 
causation  might  be  connected  with  infected  food,  but 

341 


342    DISEASES   OF    THE   NERVOUS   SYSTEM 

further  investigation  showed  that  there  was  no  ground 
for  this  belief.  Careful  consideration  was  next  given  to 
the  question  whether  the  symptoms  were  another  mani- 
festation of  the  group  known  as  the  Heine-Medin  dis- 
ease, of  which  acute  poliomyelitis  is  the  best-known 
variety,  but  sufficient  differences  were  found  in  the  age- 
incidence,  seasonal  prevalence,  course,  duration  and 
termination,  as  well  as  in  the  localization  of  the  paralyses, 
to  make  it  clear  that  the  condition  is  not  identical  with 
that  of  acute  poliomyelitis.* 

The  exact  nature  of  the  infection  has  not  yet  been 
determined,  but  it  appears  probable  that  the  patho- 
genic organisms,  like  those  of  cerebro-spinal  fever  and 
acute  poliomyelitis,  are  frequently  present  in  persons 
who  show  no  clinical  signs  of  disease  until  their  resist- 
ance is  lowered  or  the  virulence  of  the  organism  is 
increased. 

Mcintosh  and  Turnbull|  and  others  have  succeeded 
in  producing  the  disease  in  monkeys  by  inoculation  of 
brain  emulsion  from  a  fatal  case  in  man. 
It  >  Symptoms. — The  length  of  the  incubation  period 
is  uncertain  and  appears  to  be  variable.  The  prodromal 
period,  during  which  such  symptoms  as  lethargy,  head- 
ache, giddiness  and  diplopia,  with  vomiting,  diarrhoea, 
and  general  sensations  of  fatigue,  may  occur  before  the 
more  acute  stage  of  the  disease  is  reached,  commonly 
ranges  from  one  to  seven  days  but  may  last  as  long  as 
three  weeks  (MacNalty).  The  acute  stage  is  character- 
ized by  the  onset  of  stupor,  delirium,  and  other  symp- 
toms which  differ  according  to  the  intensity  of  the 
infection  and  the  locality  of  the  nervous  lesions  accom- 
panying it. 

It  will  thus  be  seen  that  the  symptoms  differ 
very  considerably  in  different  cases.  On  the  one  hand 
there  are  the  abortive  cases,  in  which  there  may  be 
nothing  more  than  a  vague  illness  characterized  by 
apathy,  sleepiness,  and  slight  pyrexia,  associated  with 
some  temporary  paresis  of  ocular  muscles  ;  while  on  the 

*Iirit.  Jouni.  Expcr.  raih.^  April,  1920.  f  Ibid. 


ENCEPHALITIS    LETHARGICA  343 

other  hand  there  are  the  severe  cases  accompanied  by 
extreme  prostration,  coma,  paresis  of  various  cranial 
nerves,  and  other  signs  of  severe  damage  to  the  nervous 
system. 

The  onset  may  be  gradual  or  rapid.  In  the  early 
stages  of  a  typical  case,  conjunctival  and  pharyngeal 
catarrh,  headache,  giddiness,  muscular  pains,  gastric 
and  intestinal  disturbance  (especially  constipation), 
together  with  some  degree  of  pyrexia,  are  all  commonly 
found,  but  the  most  characteristic  symptom  is  that  of 
lethargy,  which  tends  to  increase  in  depth.  In  some 
instances  it  does  not  amount  to  more  than  an  over- 
whelming desire  to  fall  asleep  at  inconvenient  times  and 
places,  while  in  others  it  deepens  into  a  state  of  coma 
from  which  the  patient  cannot  be  roused. 

As  the  illness  progresses  the  general  symptoms  of 
bodily  weakness  become  more  pronounced  and  the 
features  assume  a  striking  wooden,  mask-like  expression, 
somewhat  similar  to  that  seen  in  the  later  stages  of 
paralysis  agitans.  In  the  more  severe  cases  the  patient 
becomes  delirious,  the  excreta  are  passed  unconsciously, 
the  breathing  becomes  stertorous  and  altered  in 
rhythm,  the  circulation  shows  signs  of  failure,  and 
finally,  if  the  disease  causes  a  fatal  termination,  death 
takes  place  usually  towards  the  end  of  the  third  week 
of  the  illness. 

Side  by  side  with  these  general  symptoms  there  are 
various  paralyses  depending  for  their  characteristics  on 
the  locality  of  the  brain  attacked. 

Of  the  cranial  nerves,  paresis  of  the  third,  as  shown 
by  ptosis  and  limitation  of  movements  of  the  eyeball,  is 
most  commonly  found,  but  any  of  the  other  nerves  may 
also  be  affected,  and  such  symptoms  as  deafness,  paresis 
of  the  soft  palate,  and  weakness  of  the  tongue  are  all 
met  with,  as  also  are  signs  of  disturbance  of  the  vagus. 
Optic  neuritis,  due  apparently  to  rise  of  intracranial 
pressure  from  exudation,  has  also  been  observed.  When 
the  cerebral  cortex  is  affected  paralyses  of  monoplegic, 
hemiplegic,  and  diplegic  distribution,  and  disturbances 


Mi    DISEASES   OF    THE   NERVOUS   SYSTEM 

of  special  senses  such  as  hemianopia,  are  among  the 
symptoms  likely  to  be  found,  while  disease  of  the  corpus 
striatum  will  be  associated  with  tremors,  rigidity,  and 
involuntary  movements.  In  some  cases  the  occurrence 
of  rhythmical  muscular  contractions  (so-called  "  myo- 
clonus ")  have  been  observed.  Lesions  of  the  medullary 
region  are  accompanied  by  failure  of  the  heart  and 
circulation. 

Prognosis. — The  outlook  in  cases  of  any  degree  of 
severity  is  one  of  danger.  Improvements  are  often 
followed  by  relapses,  so  that  the  patient,  even  when 
seemingly  well  on  the  way  to  recovery,  cannot  be  con- 
sidered as  entirely  out  of  danger. 

Convalescence  is  slow,  and  influenced  largely  in  its 
course  by  the  nature  of  the  nervous  lesions,  some  of 
which  may  be  of  a  more  or  less  permanent  and  crippling 
nature.  Cranial  nerve  palsies  may,  for  instance,  persist, 
or  there  may  be  some  degree  of  hemiplegia  remaining, 
but  among  the  most  striking  results  of  a  more  lasting 
nature  are  the  involuntary  movements  of  limbs  and  the 
rigidity  and  tremors  of  paralysis  agitans  type,  both 
varieties  apparently  being  due  to  disease  in  the  region 
of  the  corpus  striatum.  The  involuntary  movements 
which  may  occur  in  the  face,  arm,  and  leg  resemble  in 
their  general  characters  the  athetosis  met  with  after 
cases  of  hemiplegia.  {See  p.  399.)  The  movements  are 
usually  most  prominent  in  the  arm,  and  may  either  be 
confined  to  the  hand  or,  as  frequently  happens,  the  whole 
limb  may  be  thrown  about  irregularly  or  move  with  a 
kind  of  jerky  regularity. 

The  cases  resembling  the  symptoms  met  with  in 
advanced  stages  of  paralysis  agitans  are  very  striking, 
and  are  accompanied  by  the  weakness,  rigidity,  and 
tremors  so  characteristic  of  that  disease.  The  general 
attitude  of  the  patient  is  well  shown  in  Fig.  89,  which 
should  be  compared  with  the  illustration  of  the  attitude 
in  a  case  of  paralysis  agitans  (Fig.  Ill,  p.  455). 

The  general  tendency  in  these  cases  is  for  some 
improvement  slowly  to  take  place,  but  in  many,  more 


ENCEPHALITIS    LETHARGICA 


345 


especially  in   older  people,  the   patient   is   to  a  large 
extent  permanently  crippled. 

Morbid  anatomy. — The  appearances  of  the  brain 
after  death  have  been 
found  to  vary  a  good 
deal  in  different  cases. 
Farquhar  Buzzard  and 
Greenfield*  have  investi- 
gated numerous  fatal 
cases  and  found  the  most 
common  conditions  pre- 
sent during  the  early 
stages  to  be  (1)  vascular 
congestion,  (2)  evidence 
of  toxic  degeneration  of 
nerve  cells,  (3)  prolifera- 
tion of  the  mesoblastic 
cells  of  the  vesse'-  walls 
and  infiltration  of  the 
nervous  tissue  with  these 
cells,  (4)  small-celled  in- 
filtration of  the  Virchow- 
Robin  spaces,  and  (5) 
glial  proliferation. 

Venous  thrombosis 
and  haemorrhages  were 
also  found  by  Buzzard 
and  Greenfield  in  a  cer- 
tain number  of  cases ; 
in    some    instances    the 


haemorrhages  occurred  as 
rings  round  congested 
vessels,  and  in  others  they 
were  the  results  of  in- 
farction. 

Diagnosis. — In  the  early  stages,  when  vague 
symptoms  are  accompanied  by  pyrexia,  the  illness  may 
be  indistinguishable  from  influenza  unless  some  charac- 

^  Brain,  vol.  xlii.,  pt.  iv.,  li>19. 


Fig.  89.— Paralysis  agitans  attitude 
after  encephalitis  lethargica. 


346     DISEASES  OF  THE  NERVOUS  SYSTEM 

teristic  symptoms  such  as  ocular  palsies  or  excessive 
drowsiness  arise. 

An  inflammatory  lesion  in  the  course  of  the  motor 
fibres  may  give  rise  to  hemiplegia,  while  disturbances  of 
the  sensory  fibres  may  be  accompanied  by  hemianses- 
thesia  or  hemianopia,  all  of  which  are  apt  to  be  ascribed 
to  results  of  primary  vascular  changes,  such  as  throm- 
bosis or  hcemorrhage,  unless  the  possibility  of  encepha* 
litis  is  kept  in  mind.  Meningitis  and  cerebral  tumour 
may  also  be  simulated  by  the  severe  headache  and  other 
signs  of  increased  intracranial  pressure,  including  optic 
neuritis,  that  sometimes  occur  from  the  exudation  of 
serous  or  bloodstained  fluid,  and  if  they  persist  after  the 
acute  signs  of  general  infection  have  subsided,  the  clinical 
signs  may  seem  to  be  those  of  a  serous  meningitis  or 
(when  the  exudation  is  circumscribed)  of  a  cerebral 
tumour  {see  p.  326). 

Treatment. — Up  to  the  present  there  is  no  specific 
remedy  for  the  disease,  and  treatment  has  mainly  to 
be  directed  towards  relief  of  symptoms  and  maintenance 
of  nutrition.  In  selected  cases  lumbar  puncture  is 
sometimes  followed  by  relief  of  head  symptoms,  and 
may  be  repeated  as  thought  necessary,  provided  the 
tension  of  the  cerebro-spinal  fluid  is  raised. 

CEREBRAL  ABSCESS    (SUPPURATIVE 
ENCEPHALITIS) 

Etiology. — The  source  of  the  infection  in  a  case 
of  cerebral  abscess  may  be  in  the  skull  or  in  some 
distant  part  of  the  body.  Chronic  suppuration  of  the 
middle  ear  is  by  far  the  most  common  cause,  but  in- 
fection may  be  carried  equally  well  from  other  neigh- 
bouring parts,  and  Sir  StClair  Thomson  has  shown  that 
diseases  of  the  nasal  sinuses  are  more  important  in  this 
respect  than  had  generally  been  supposed. 

Necrosis  of  any  of  the  bones  of  the  skull,  arising 
from  injury,  syphilis,  or  other  diseases,  may  be  followed 
by  an  abscess. 

From  more  distant  parts  the  infection  is  particularly 


CEREBRAL   ABSCESS  347 

apt  to  follow  empyemata,  bronchiectasis,  and  pulmonary 
abscess. 

At  other  times  the  cerebral  suppuration  is  but  part 
of  a  generalized  pyaemia,  in  which  case  the  abscesses 
are  often  multiple. 

The  posterior  parts  of  the  temporo-sphenoidal  lobes, 
and  the  cereb  ellum,  from  their  proximity  to  the  bones  of 
the  ear,  are  th  e  areas  in  which  abscesses  most  commonly 
occur ;  less  often  the  occipital  and  parietal  lobes  are 
infected.  When  the  infective  matter  is  brought  from 
distant  parts  the  left  side  of  the  brain  is  rather  more 
likely  to  be  attacked  than  the  right. 

Pathology. — When  the  abscess  follows  diseased 
bone  the  dura  mater  over  the  necrosed  area  is  usually 
thickened,  but  the  superficial  portion  of  the  cortex 
generally  appears  healthy  to  the  naked  eye,  and  at  a 
variable  distance  beneath  its  surface  is  situated  the 
collection  of  pus.  The  channel  by  which  the  infection  is 
conveyed  to  the  brain  is  probably  not  always  the  same ; 
sometimes  it  is  by  lymphatics,  and  sometimes  by  veins. 

If  the  abscess  has  existed  for  some  time,  it  is  likely 
to  be  enclosed  by  a  definite  capsule,  the  result  of  organized 
products  of  inflammation  in  the  neuroglial  tissue  ;  often, 
however,  there  is  no  such  boundary,  and  a  large  area 
of  brain  tissue  may  be  permeated  by  pus,  Hke  water 
in  a  sponge. 

The  colour  of  the  pus  is  usually  a  greenish  hue,  and 
the  odour  is  generally  foul.  Streptococci  and  staphylo- 
cocci can  often  be  found,  as  also,  occasionally,  may 
tubercle  bacilU  and  other  specific  micro-organisms. 

Symptoms. — The  symptoms  of  an  abscess,  as  might 
be  expected,  resemble  to  some  extent  those  of  tumour, 
and  they  may  be  divided  in  a  similar  fashion  into  general 
and  local  (p.  352). 

Sometimes  an  abscess  may  exist  for  an  indefinite 
time  without  producing  any  symptoms  at  all ;  and 
in  chronic  cases,  latent,  acute,  and  terminal  stages  may 
be  well  defined.  In  many  there  is  a  "  latent  period  " 
of    some    length.     When    symptoms    arise,    headache, 


348    DISEASES   OF   THE   NERVOUS  SYSTEM 

vomiting,  and  optic  neuritis  may  all  be  present ;  but, 
compared  with  tumours,  the  pressure  produced  by  an 
abscess  is  not  often  so  great,  and  thus  optic  neuritis  is 
not  so  frequently  found,  nor  is  it  usually  so  intense,  and 
for  the  same  reason  the  vomiting  and  headache  may  be 
less  troublesome.  On  the  other  hand,  mental  dullness 
is  generally  more  pronounced  than  in  the  case  of  tumours. 
The  slow  pulse  is  present  in  both.  In  the  terminal  stages 
there  may  be  signs  of  septic  absorption  which  do  not 
exist  in  most  cases  of  tumour,  e.g.  a  quickened  pulse 
and  an  irregular  temperature,  with  occasional  rigors  ; 
and  in  the  last  stages  of  the  disorder  there  may  be 
delirium  and  coma.  The  onset  of  these  more  general 
symptoms  is  suggestive  of  the  coincidence  of  meningitis. 

In  many  cases  the  septic  symptoms  are  rendered 
more  intense  by  absorption  of  septic  matter  from  other 
regions  besides  that  of  the  actual  abscess. 

Left  to  itself,  an  abscess  which  is  giving  rise  to  acute 
symptoms  will  cause  death,  either  by  direct  pressure 
on  vital  parts,  or  after  rupture  into  one  of  the  ventricles, 
or  by  infection  of  the  membranes  and  the  supervention 
of  general  meningitis. 

The  symptoms  upon  which  the  diagnosis  of  the  posi- 
tion of  the  abscess  depends  are  those  which  are  discussed 
in  dealing  with  the  localization  of  tumours  (p.  355).  In 
the  temporo- sphenoidal  lobe  there  will  be  the  likelihood 
of  sensory  aphasia,  or,  if  the  abscess  is  in  the  forepart 
of  the  lobe,  there  may  be,  as  in  the  instance  narrated 
elsewhere  (p.  365),  a  disturbance  of  the  sense  of  smell. 
If  the  pressure  is  considerable  the  motor  fibres  may 
suffer,  and  there  will  be  signs  of  hemiplegia  on  the 
opposite  side  of  the  body. 

When  the  pus  is  in  the  lateral  lobe  of  the  cerebellum, 
vertigo,  nystagmus,  paresis  of  the  external  rectus,  with 
conjugate  or  skew  deviation  of  the  eyes,  are  among 
the  prominent  localizing  symptoms.  One  of  the  greatest 
aids  in  the  diagnosis  of  the  site  of  the  abscess  is  the 
position  of  the  primary  suppuration,  to  which  the  abscess 
is  secondary ;  the  two  are  nearly  always  in  close  proxi- 


CEREBEAL   ABSCESS  349 

mity,  and  in  the  majority  of  the  cases  the  primary  seat 
of  disease  is  connected  with  the  ear. 

Diagnosis. — The  principal  local  conditions  from 
which  abscess  has  to  be  distinguished  are  meningitis, 
suppuration  in  the  mastoid  cells,  and  thrombosis  of  cerebral 
sinuses  ;  the  difficulty  in  discriminating  between  these 
several  conditions  is  increased  by  the  fact  that  they 
may  all  be  present  at  the  same  time. 

If  the  disease  is  confined  to  the  mastoid  processes, 
the  pain,  redness,  tenderness,  and  other  symptoms  will 
be  unaccompanied  by  any  definite  signs  of  implication 
of  the  brain.  There  will  not  be  mental  dullness,  vomit- 
ing, or  optic  neuritis,  nor  will  there  be  signs  of  pressure 
on  any  particular  set  of  fibres.  Moreover,  the  tempera- 
ture will  usually  be  raised  instead  of  being  subnormal ; 
and  rigors  are  common. 

It  is  thus  more  often  by  a  process  of  exclusion  than 
otherwise  that  the  diagnosis  is  made.  In  the  same 
manner,  careful  consideration  has  to  be  given  to  the 
possibility  of  an  abscess  complicating  sinus  thrombosis, 
the  symptoms  of  which  are  detailed  in  Chapter  XLI., 
p.  409. 

In  meningitis  the  signs  are  more  generalized,  and 
the  cranial  nerves  are  more  likely  to  sufier.  The  patient 
tends  to  assume  a  curled-up  position,  is  irritable,  shuns 
the  light,  and  objects  to  being  touched  ;  convulsions 
may  also  be  present.  The  diagnosis  may  be  aided  by 
performing  lumbar  puncture,  for  the  cerebro-spina 
fluid  is  liable  to  contain  micro-organisms  in  the  case 
of  meningitis  but  not  in  abscess.  Leucocytosis  of  the 
blood  may  be  present  in  both  cases. 

A  change  in  the  percussion  note  over  the  side  of  the 
head  in  which  the  abscess  is  situated  has  sometimes  been 
observed.  Tenderness  over  the  skull  in  the  vicinity  of 
the  abscess  is  another  sign  that  has  occasionally  been 
noted. 

Prognosis  and  treatment. — ^The  prognosis  in  the 
case  of  a  patient  with  a  cerebral  abscess  must  always 
be  doubtful.     When  there  are  several  abscesses  which 


350    DISEASES    OF    THE   NEEVOUS   SYSTEM 

are  merely  a  part  of  some  general  pyaemic  condition  the 
outlook  is  hopeless  ;  but  when  there  is  only  one  abscess 
the  final  result  depends  upon  the  possibility  of  opening 
and  efficiently  draining  it. 

If  this  can  be  accomplished — ^as  is,  fortunately,  often 
possible  when  the  abscess  arises  from  disease  of  the 
middle  ear  and  the  mastoid — ^the  chances  of  recovery 
are,  on  the  whole,  fairly  good.  It  is  obvious  that  no 
medicinal  treatment  can  give  relief,  and  therefore, 
when  once  the  diagnosis  is  established,  there  should 
be  no  time  lost  in  resorting  to  surgical  measures. 


CHAPTER   XXXVII 

INTRACRANIAL  TUMOURS 

Vai'ieties. — The  growths  that  occur  in  the  brain  com- 
prise infective  granulomata,  carcinomata,  sarcomata, 
gliomata,  benign  tumours  of  various  kinds,  and  cysts 
of  simple  and  of  parasitic  origin. 

With  the  exception  of  carcinomata,  the  growths  tend 
to  occur  before  rather  than  after  middle  life,  and  are 
frequently  found  in  children.  Of  the  infective  granulo- 
mata, tuberculous  and  syphilitic  growths  are  the  most 
common ;  they  are  frequently  multiple.  Tuberculous 
growths  are  chiefly  met  with  in  the  young;  in  them 
they  show  a  predilection  for  the  cerebellum,  and  may 
sometimes  subside  into  quiescence. 

Gummata  nearly  always  arise  from  the  cerebral 
membranes,  and  only  rarely  are  found  deep  in  the  sub- 
stance of  the  brain.  The  commonest  positions  in  which 
to  find  them  are  the  base  and  cortex  of  the  cerebrum, 
the  structures  of  the  cerebellum  being  invaded  com- 
paratively seldom.  Gummata  are  frequently  multiple 
and  accompanied  by  a  certain  degree  of  meningitis, 
which  causes  further  damage  to  the  nerve  substance 
arouud. 

Actinomycosis  has  occasionally  been  found  within 
the  skull. 

Carcinomata  generally  occur  in  later  life,  and  are 
always  secondary  to  a  growth  elsewhere.  According 
to  Handley,*  the  cerebellum  shows  a  special  liability  to 
be  attacked. 

*  *'  Cancer  of  the  Breast,"  1906. 
351 


352    DISEASES  OF  THE  NERVOUS   SYSTEM 

Sarcomata  are  either  primary  or  secondary,  and 
may  arise  in  any  part  of  the  brain.- 

Endotheliomata  grow  from  the  membranes  of  the 
brain.  They  differ  considerably  from  one  another  in 
the  intensity  of  their  malignancy. 

The  fsammomata,  which  contain  earthy  matter  and 
cholesterin,  belong  to  this  last  group; 

Gliomata  originate  in  the  neuroglia.  They  infiltrate 
rather  than  compress  the  brain  tissue.  Owing  to  their 
close  resemblance  to  the  cerebral  substance  their  bound- 
aries are  difficult  to  define,  and  their  general  appear- 
ance is  often  one  of  hypertrophy  of  a  part  of  the 
brain.  These  tumours  are  usually  single,  and  do  not 
give  rise  to  secondary  deposits;  they  are  prone  to 
undergo  degeneration,  and  occasionally  they  become 
quiescent. 

The  benign  tumours  are  of  comparatively  rare  occur- 
rence. 

Neuro-fibromata  occasionally  grow  from  the  sheaths 
of  the  cranial  nerves.  Adenomata  and  cystic  degenerations 
may  arise  in  connection  with  the  pituitary  body. 

Cholesteatomata,  lipomata,  and  angiomata  occur  oc- 
casionally, and  sometimes  an  osteoma  grows  inwards 
and  compresses  the  bi-ain. 

Parasitic  cysts  occur  in  connection  with  the  Tcenia 
echinococcus  and  Cysticercus  cellulosce,  and  simple  cysts 
are  also  sometimes  found. 

An  intracranial  aneurysm  may  give  rise  to  symptoms 
of  a  tumour,  according  to  its  size  and  position. 

Symptoms. — There  are  certain  symptoms  which 
may  be  associated  with  any  intracranial  tumour,  irrespec- 
tive of  its  nature  or  situation,  while  others  are  peculiar 
— or  accidental,  so  to  speak — to  the  position  in  which 
the  tumour  happens  to  be  placed.  Thus  the  symp- 
toms may  be  grouped  under  the  headings  of  general 
and  local. 

The  general  symptoms  are  mainly  the  result  of 
increased  intracranial  pressure,  and  they  include  head- 
ache, vertigo,  vomiting,  optic  neuritis,  slow  pulse,  sub- 


INTRACRANIAL    TUMOURS  355 

normal  temperature,  with  possibly  a  general  convulsion, 
and  some  mental  failure. 

It  is  hardly  necessary  to  say  that  frequently  not 
all  of  these  are  present  in  any  given  case,  and  that  the 
absence  of  one  or  more  of  them  by  no  means  necessarily 
invalidates  the  diagnosis. 

Headache. — Headache  is  one  of  the  most  frequent 
symptoms  of  intracranial  tumours.  After  it  has  once  set 
in  it  seldom  disappears,  but  its  intensity  is  nevertheless 
liable  to  considerable  fluctuations.  The  usual  history 
is  that  of  a  dull  ache  with  agonizing  exacerbations. 
When  the  growth  is  due  to  syphilis  the  exacerbations 
are  most  notable  during  the  night,  and  there  is  often 
some  localized  tenderness,  which  can  seldom  be  satis- 
factorily demonstrated  in  association  with  other  kinds 
of  tumour. 

With  the  possible  exception  of  the  syphilitic  cases, 
the  position  of  the  headache  cannot  in  itself  be  relied 
upon  to  any  extent  as  a  guide  to  the  position  of  the 
tumour,  though,  in  conjunction"  with  other  symptoms, 
the  seat  of  the  pain  may  be  strongly  suggestive. 

In  cerebellar  tumours,  for  example,  the  pain  is  often 
felt  most  intensely  in  the  occipital  region,  and,  if  the 
other  signs  are  in  keeping,  this  localization  of  pain 
may  be  given  a  certain  value  ;  but  it  sometimes  happens 
that  the  headache  of  tumours  in  the  posterior  part  of 
the  brain  is  chiefly  frontal  in  distribution,  and,  con- 
versely, that  of  frontal  tumours  may  be  mainly  felt  at 
the  back  of  the  head. 

Vomiting. — Vomiting  is  a  common  symptom.  Some- 
times it  is  preceded  by  nausea,  while  at  other  times 
the  contents  of  the  stomach  are  suddenly  brought  up 
without  any  warning.  It  may  occur  without  any  definite 
relation  to  taking  food.  Vomiting  is  liable  to  be  very 
pronounced  in  tumours  situated  beneath  the  tentorium, 
where  their  growth  is  confined  and  causes  a  rapid  rise 
of  pressure. 

Of  tic  neuritis. — Of  all  the  signs  of  intracranial  tumour, 
optic  neuritis  is  probably  the  most  important.     It  occurs 

X 


354    DISEASES   OF   THE   NERVOUS   SYSTEM 

in  a  large  percentage  of  cases,  and,  like  the  vomiting, 
is  apt  to  be  very  intense  with  subtentorial  growths. 
The  neuritis  is  sometimes  more  advanced  on  one  side 
than  the  other,  and  such  inequality  has  a  certain  degree 
of  value  in  localization  by  suggesting  that  the  growth  is 
in  that  half  of  the  brain  which  corresponds  to  the  in- 
flammation of  longer  standing.  Too  much  dependence 
must  not,  however,  be  placed  upon  it.* 

It  may  also  be  said,  roughly  speaking,  that  the 
rapidity  of  the  neuritis  tends  to  be  somewhat  propor- 
tionate to  the  rate  of  growth  of  the  tumour,  though 
there  must  necessarily  be  many  exceptions  to  this 
statement. 

Temporary  attacks  of  dimness  or  loss  of  vision, 
wliich  are  usually  accompanied  by  exacerbations  of  head- 
ache and  vertigo,  are  occasionally  met  with  during  the 
course  of  intracranial  tumours,  more  especially  of  those 
of  the  cerebellum,  and  are  regarded  as  being  due  to  a 
temporary  strangulation  of  the  optic  nerve  or  to  pressure 
of  the  infundibulum  upon  the  optic  chiasma  (Leslie 
Paton). 

The  slow  pulse  and  the  low  temperature  are  both 
variable  symptoms,  and  are  produced  by  the  increased 
intracranial  pressure. 

General  convulsions,  though  important  when  they 
occur  as  an  early  symptom,  are  distinctly  uncommon, 
but,  unless  their  possible  connection  with  tumours  be 
remembered,  they  may  easily  be  diagnosed  as  epilepsy 
until  suspicions  are  aroused  by  the  appearance  of  other 
signs.  The  greatest  safeguard  against  overlooking  a 
tumour  consists  in  systematically  examining  the  optic 
discs. 

Mental  changes  sometimes  occur  quite  apart  from  the 

disturbance  of  any  special  part  of  the  brain,  and  are 

then  to  be  attributed  chiefly  to  the  rise  of  pressure 

within  the  skull. 

■"  Of  50  cases  aualysed  by  C.  E.  Beevor,  iu  19  the  ueuritis  was 
more  intense  on  the  side  of  the  lesion,  in  11  it  was  more  marked 
on  the  side  opposite  the  lesion,  and  in  1.'3  it  was  equal  on  the  two 
sides.     In  the  remaining  7  there  was  no  neuritis. 


INTRACEANIAL  TUMOURS  355 

Local  symptoms  are  considered  in  the  following 
section  : — 

Localization  of  Intracranial  Tumours 

The  abiUty  to  localize  the  position  of  intracranial 
tumours  depends  first  upon  a  knowledge  of  the  ana- 
tomical seat  of  those  functions  of  the  brain  which 
are  disturbed.  Hence  it  is  only  possible  to  localize 
tumours  accurately  when  they  are  situated  in  certain 
regions  of  the  brain,  for  when  they  are  in  parts  whose 
functions  are  unknown — ^the  so-called  "  silent  areas  " 
— the  general  symptoms  are  accompanied  by  no  special 
local  disturbances  by  which  the  position  of  the  tumour 
can  be  defined. 

Another  source  of  difficulty  lies  in  separating  the 
symptoms  produced  in  the  immediate  yicinity  of  the 
tumour  from  those  which  arise  some  distance  off  as 
the  result  of  indirect  pressure  effects. 

The  value  of  local  symptoms  also  varies  according 
to  the  date  of  their  appearance.  They  are  most  reliable 
when  their  advent  coincides  with  or  shortly  follows  the 
appearance  of  the  general  symptoms,  for  when  local 
symptoms  appear  only  a  long  time  after  the  onset  of 
general  symptoms,  the  chances  are  many  (as  shown 
by  Collier)  that  they  may  not  then  point  to  the  primary 
position  of  the  growth,  but  rather  represent  the  results 
of  such  secondary  changes  as  hydrocephalus,  meningitis, 
vascular  lesions,  and  metastatic  deposits ;  and,  further, 
as  the  same  author  has  also  shown,  the  long-continued 
absence  of  local  signs,  when  the  general  signs  are  well 
marked,  is  in  itself  of  importance  in  suggesting  that 
the  tumour  is  situated  above  rather  than  below  the 
tentorium,  since  in  the  latter  case  localizing  symptoms 
generally  appear  early; 

Assuming  that  the  signs  of  a  tumour  are  present,  the 
various  effects,  according  to  the  position  of  the  growth, 
may  now  be  considered. 

1.  Tumours  interfering  with  the  motor  tracts. 
— "When  the  presence  of  a  tumour  is  associated  with  loss 


356    DISEASES   OF   THE   NERVOUS   SYSTEM 


of  voluntary  movements  we  may  assume  that  the  fibres 
which  conduct  the  motor  impulses  are  interrupted  at 
some  point  in  their  course  between  the  motor  area  of 
the  cerebral  cortex  and  the  decussation  at  the  medulla, 
and  the  different  effects  may  be  considered  according 
as  the  growth  is  situated  in  (i.)  the  motor  area  of  the 


Anus  ffyd.^ina. 


Abdom&n 

Chest 


Fin^rs 
if  thumb. 


OpeninC 
of  jaw 


VoccbL 
cords 


Sulcus  centralis 

Ma^stication 


CiS.rfey. 


Fig.  90.— Diagram  showing  the  situation  of  the  motor  areas  in  the 
cortex.     {Slier ringtail  and  Grunbanm.) 

cortex,  (ii.)  the  region  of  the  internal  capsule,  (iii.)  the 
crus  cerebri,  or  (iv.)  the  pons  Varolii. 

i.  Tumours  of  the  motor  area  of  the  cortex. — The  extent 
of  the  motor  area  of  the  cortex  is  seen  from  Fig.  90, 
which  depicts  the  arrangement  found  by  Sherrington 
and  Griinbaum  in  the  highest  apes,  and  which  pre- 
sumably closely  conforms  to  that  of  the  human  brain. 
It  will  be  noticed  that  there  are  two  important  modi- 


TUMOUES    OFJ  MOTOR  ^AREAl        [357 

fications   of   the   plan  which   was    formerly  considered 
correct,  viz. :  ^  I 

(a)  The  whole  of  the  motor  area  is  situated  in  front 
of  the  fissure  of  Rolando  and  is  confined  to  the  ascending 
frontal  convolution. 

(b)  The  Representation  for  movements  of  the  trunk 
is  placed  between  those  for  the  arm  and  leg,  instead 
of  being  situated  on  the  margin  of  the  great  longitudinal 
fissure.- 

Now,  if  a  tumour  be  situated  in  this  motor  area,  it 
is  obvious  that  at  first  it  will  be  likely  to  affect  only 
a  small  portion  of  the  convolution,  and,  as  a  general 
rule,  the  weakness  is  for  a  time  confined  to  one  limb  or  to 
the  face,  i.e.  there  is  a  monoplegia,  usually  of  slow  develop- 
ment, which  later  on  extends  to  a  hemiplegia  as  the  grow- 
ing edges  of  the  tumour  spread  into  the  neighbouring 
areas.  At  the  same  time  that  the  growth  is  reducing 
the  power  of  movement,  it  is  also  irritating  the  motor 
cells  in  its  immediate  vicinity.  The  result  of  this  irrita- 
tion is  seen  in  convulsions,  which  begin  in  a  group  of 
muscles  corresponding  to  the  irritated  area  of  the  cor- 
tex, and  subsequently  spread  with  some  deliberation  to 
other  parts  of  the  limb,  when  they  may  either  subside 
without  loss  of  consciousness  or  develop  into  a  general 
convulsion.  The  significance  of  these  localized  convul- 
sions as  the  indicators  of  the  particular  area  of  cortex 
that  is  diseased  was  first  pointed  out  by  Hughlings 
Jackson,  from  which  circumstance  they  are  known  as 
JacJcsonian  epilepsy. 

Localized  con^nilsions  are  not  in  themselves  pathog- 
nomonic of  the  presence  of  gross  cerebral  lesions,  for 
they  are  frequently  met  with  in  the  course  of  ordinary 
epilepsy.  Their  significance  is  the  same  in  both  cases, 
inasmuch  as  they  indicate  the  part  of  the  brain  in  which 
the  epileptic  discharge  begins ;  but,  where  the  general 
signs  point  to  the  presence  of  a  gross  lesion,  their  occur- 
rence is  of  the  greatest  importance  in  enabHng  its  position 
to  be  determined.  Lastly,  in  addition  to  loss  of  power, 
a  tumour  of  the  motor  cortex  is  sometimes  accompanied 


358    DISEASES    OF    THE   NERVOUS   SYSTEM 

by   some   disturbance   of  sensation,   due    probably   to 
pressure  on  the  ascending  parietal  lobe.     The  sensory 


Monoplegia 
with  increased 
reflexes 

Jacksouian 
epilepsy 

Possibly  some 
disturbance  of 
sensation 


Fig.  91.— Scheme  of  the  principal  localizing  symptoms  produced  by 
a  tumour  in  the  cortical  motor  area  for  the  arm. 


TUMOURS   OF   MOTOR   AREA 


359 


disturbance  from  a  cortical  lesion  will  not  be  in  the  form 
of  loss  of  crude  recognition  of  touch,  pain,  heat  and  cold, 


Hemianopia  - 


Tumuur 
pressing 
upon  the 
left  internal 
capsule 


Hemiplegia  ]   / 
and  [/_ 

heniian- 
aesthesia 


\ 


\ 


Fig-  92.— Scheme  of  the  principal  localizing  symptoms  produced  by 
a  tumour  pressing  on  the  internal  capsule. 


360    DISEASES   OF   THE  NERVOUS   SYSTEM 

but  will  show  itself  by  want  of  discriminative  power  as 
described  under  Tumours  of  the  Parietal  Eegion  (p.  363). 
To  sum  up,  the  main  signs  upon  which  reliance  can  be 
placed  in  the  localization  of  a  tumour  in  the  area 
of  the  motor  cortex  are  monoplegia  and  Jacksonian 
epilepsy,  with  j)erhaps  some  disturbance  of  sensation. 
(Fig.  91.) 

ii.  Tumours  in  the  region  of  the  internal  capsule. — 
At  the  internal  capsule  the  motor  fibres  are  packed 
closely  together,  and  a  tumour  pressing  upon  them  is 
most  likely  to  impair  the  functions  of  all,  and  so  to 
cause  a  hemiplegia  of  slow  onset.  At  the  same  time 
there  may  be  a  certain  amount  of  hemiansesthesia,  since 
the  sensory  fibres  occupy  the  posterior  third  of  the 
posterior  limb  of  the  capsule,  but  in  slow-growing  tu- 
mours this  is  often  not  noticeable.  The  fibres  of  vision 
from  the  occipital  lobes  may  also  be  interrupted  as  they 
pass  across  the  hindmost  part  of  the  internal  capsule, 
in  which  case  there  will  be  hemianopia.  There  will  be 
no  Jacksonian  convulsions,  though  there  may  occasionally 
be  a  generalized  fit  due  to  the  effects  of  general  pressure 
on  the  cortex. 

The  main  localizing  symptoms  of  a  tumour  pressing 
on  the  internal  capsule  are,  therefore,  hemiplegia,  he^ni- 
ancBsthesia,  and  Jiefnianopia.     (Fig.  92.) 

iii.  Tumours  of  the  crus  cerebri. — There  will  be  the 
same  tendency  for  a  tumour  of  the  crus  to  give  rise  to 
hemiplegia  and  hemiansesthesia  of  the  opposite  side  as 
there  is  in  the  case  of  a  tumour  situated  in  the  internal 
capsule,  with  the  probable  addition  of  paresis  of  certain 
cranial  nerves,  more  especially  of  the  3rd,  from  pressure 
on  its  nucleus  or  on  the  fibres  springing  directly  from 
it.  This  gives  rise  to  a  "  crossed  "  paralysis,  i.e.  the 
face,  arm,  and  leg  are  paralysed  on  the  one  side  (the  side 
opposite  the  tumour),  and  the  ocular  muscles  supplied 
by  the  3rd  nerve  are  paralysed  on  the  other  side  (the 
same  side  as  the  tumour). 

In  addition,  other  cranial  nerves,  e.g.  the  4th,  5th, 
and  Gth,  may  be  pressed  upon  if  the  tumour  is  a  large  one. 


TUMOURS    IN    PONS    VAROLII 


361 


Tremors  have  also  been  observed  in  these  cases. 

Thus  the  main  diagnostic  points  of  a  tumour  in  iLc 
crus  cerebri  are  crossed  paraly- 
sis (i.e.  loss  of  power  in  the 
face,  arm,  and  leg  on  the  side 
opposite  to  the  tumour,  and 
paralysis  of  ocular  muscles, 
and  possibly  facial  muscles, 
on  the  same  side),  Jiemia^ices- 
tJiesia  and  possibly  tremors. 

iv.  Tumours  in  the  pons. — 
Tumours  which  are  situated 
in  the  pons  tend  to  cause 
symptoms  somewhat  similar 
to  those  of  the  crus,  inasmuch 
as  they  give  rise  to  hemi- 
plegia and  hemianaBsthesia  of 
the  opposite  side  and  also  to 
a  crossed  paralysis  (Figs.  93 
and  94).  In  this  instance  it 
is  the  facial  nerve  that  usually 
suffers  on  the  same  side  as 
the  tumour,  but  the  oculo- 
motor (3rd),  the  abducens 
(6th),  and  the  auditory  (8th) 
may  also  be  affected.  It  is 
also  in  a  similar  manner  pos- 
sible for  lesions  of  the  pons  to 
cause  crossed  JiemiayicBsthesia, 
by  damaging  the  fibres  of 
the  trigeminal  nerve  going  to 
the  one  side,  and  those  for 
the  arm,  trunk,  and  leg  on  the 
opposite  side.  There  may  be 
conjugate  deviation  of  the 
eyes  away  from  the  side  of 
the  tumour,  or  nystagmus  may 
be  present.     Optic  neuritis  is      ^^^p'LaitSsf  fao?aTecfed 

often  comparatively   late    in  ^^  P^e  side,  arm  and  leg 

^  *'  on  the  other. 


362    DISEASES   OF   THE   NERVOUS  SYSTEM 

making  its  appearance.  The  main  points  to  be  relied 
on  are  hemiplegia  and  hemianwsthesia  on  the  opposite 
side,  with  facial  paralysis,  deafness,  and  ocular  palsies  on 
the  same  side  as  the  tumour. 

In  the  medulla  the  motor  fibres  of  the  two  sides 
are  so  close  together  that  a  tum.our  in  their  immediate 


.VII 


Fig.  M.— Diagram  to  show  the  mechanism  of  crossed  paralysis. 
The  lesion  at  A  paralyses  the  facial  nerve,  B,  on  the  one  side 
and  the  fibres  for  the  arm  and  leg,  C,  on  the  other. 

neighbourhood  is  liable  to  cause  bilateral  paralysis, 
though  in  all  probability  the  loss  of  power  will  be  more 
marked  on  one  side  than  on  the  other. 

In  addition  to  this,  there  is  every  likelihood  of  the 
existence  of  symptoms  due  to  pressure  on  the  bulbar 
nuclei,  such  as  difficulty  in  speaking  and  swallowing, 
and  weakness  of  the  tongue.  Glycosuria  may  also  be 
found. 


TUMOURS   OF    FRONTAL   LOBES        363 

2.  Tumours  situated  in  parts  of  the  cerebral 
cortex  other  than  the  motor   area.     i.  Tumours  of 

the  frontal  lobes. — Tumours  of  the  frontal  lobes,  whether 
cortical  or  subcortical,  are  admittedly  difficult  to  diag- 
nose, since  they  may  attain  to  a  large  size  and  yet  give 
rise  to  no  localizing  symptoms. 

The  functions  of  the  frontal  lobes  are  regarded  as 
being  mainly  connected  with  the  intellect,  and  hence 
mental  disturbance,  in  conjunction  Avith  the  absence  of 
signs  that  the  tumour  is  situated  elsewhere,  is  the  chief 
condition  upon  which  reliance  must  be  placed.  Mental 
disturbances  are,  however,  often  caused  by  tumours 
in  other  parts  of  the  brain,  although  Mills,  who  has 
contributed  largely  to  this  important  subject,  considers 
that  the  conflict  of  evidence  is  more  apparent  than  real, 
and  that  it  would  disappear  if  sufficient  care  were  taken 
to  analyse  the  symptoms  and  to  exclude  those  which 
are  of  a  more  general  character  and  obviously  due  to 
widespread  irritation  from  a  tumour.  Mills  believes 
that  if  every  case  of  disease  of  the  prefrontal  lobes  (and 
more  especially  of  the  left  lobe)  were  carefully  analysed, 
some  mental  symptoms  of  a  special  character  in  con- 
nection with  the  higher  psychic  activities  would  be 
recognized. 

Grainger  Stewart  has  observed  that  the  superficial 
abdominal  reflexes  are  often  diminished  or  lost  on  the 
opposite  side  to  the  tumour,  and  that  there  is  apt  to  be 
a  fine,  rapid,  vibratory  tremor  of  the  limbs  on  the  same 
side  as  the  growth. 

As  the  tumour  grows,  the  signs  of  encroachment  on 
the  motor  areas  for  the  head  and  eyes,  the  limbs,  or  the 
motor  centre  for  speech  (Broca's  convolution)  may  give 
the  clue  to  its  position. 

ii.  Tumours  of  the  parietal  region. — A  large  number  of 
sensory  fibres  impinge  on  the  parietal  lobes,  and  a  dis- 
turbed function  of  these  is  the  chief  effect  of  a  tumour 
of  this  region. 

The  loss  of  sensation  in  these  cortical  cases,  as  already 
mentioned  (p.  359),  will  not  take  the  form  of  inability  to 


364    DISEASES   OF   THE   NERVOUS   SYSTEM 

recognize  crude  sensations  of  touch,  pain,  heat  and  cold, 
but  will  be  characterized  by  lack  of  discrimination  as 
regards  (1)  recognition  of  spatial  relations,  (2)  graduated 
responses  to  stimuli  of  different  intensity,  (3)  apprecia- 
tion of  similarity  and  difference  in  external  objects 
brought  into  contact  with  the  surface  of  the  body.  The 
form  of  discrimination  most  often  impaired  is  that  of 
knowledge  of  spatial  relations,  and  clinically  an  inability 
to  recognize  passive  movements  of  an  affected  limb  is  to 
be  regarded  as  one  of  the  principal  signs  of  disease  of 
the  sensory  area  of  the  cortex  (Head).  Additional  signs 
of  the  same  nature  are  loss  of  power  to  distinguish 
differences  in  weights  and  shapes,  the  last-named  being 
often  spoken  of  as  the  "  stereognostic  sense  "  {see 
Sensation  and  the  Cerebral  Cortex,  p.  23).  To  the 
sensory  signs  others  are  usually  added  through  the 
encroachment  of  the  tumour  on  the  parts  around. 
Thus,  if  it  extends  forwards,  there  will  be  motor 
symptoms;  if  backwards,  the  functions  of  the  angular 
gyrus  and  occipital  lobe  will  be  impaired ;  while  if 
the  disease  spreads  beneath  the  cortex,  it  may  affect 
the  motor  fibres  as  they  jysiss  through  the  corona 
radiata.  Convulsions  associated  with  tumours  of 
the  parietal  lobes  are  usually  preceded  by  sensory 
warnings. 

When  the  tumour  encroaches  on  the  occipital  lobe 
there  may  be  hemianopia.  Visual  aphasia  (word- 
blindness)  may  result  from  tumours  of  this  region 
when  they  are  situated  on  the  left  side.  Fits  beginning 
with  visual  warnings  may  also  be  associated  with 
tumours  encroaching  on  the  occipital  region. 

iii.  Tumours  of  the  occipital  lobes. — Fits  beginning  with 
visual  warnings  may  be  the  early  signs  of  tumours  of  the 
occipital  region  of  the  cortex.  There  may  also  be 
hemianopia,  i.e.  loss  of  half-vision  in  the  corresponding 
parts  of  the  two  fields  (homonymous  or  simple  hemi- 
anopia) ;  but  the  value  of  this  symptom  is,  of  course, 
frequently  lost  through  the  general  impairment  of  vision 
from  optic   neuritis.     Occasionally  only  a  quadrant  of 


TUMOURS    OF    UNCINATE    GYRUS        365 

the  usual  field  is  lost  (quadrantic  hemianopia).  If  the 
tumour  is  on  the  left  side  there  may  be  word-blindness 
with  diminished  fields  of  vision,  as  mentioned  in  the 
previous  paragraph. 

iv.  Tumours  of  the  temporo-sphenoidal  lobes. — Tumours 
of  the  temporo-sphenoidal  lobes  may  cause  deafness  on 
the  opposite  side,  and,  if  situated  on  the  left  side,  there 
may  also  be  sensory  aphasia  (word-deafness).  Auditory 
warnings  may  occur  before  a  fit,  or  hallucinations  of 
hearing  may  be  present.  Extension  of  the  tumours 
inwards  may  cause  pressure  on  the  motor  and  sensory 
fibres  for  the  opposite  limbs. 

V.  Tumours  of  the  uncinate  gyrus. — Tumours  of  the 
anterior  part  of  the  temporo-sphenoidal  lobe  are  some- 
times accompanied  by  the  "  dreamy  states  "  first  de- 
scribed by  Hughlings  Jackson. 

Sensations  of  smell  may  also  accompany  tumours 
in  this  region — a  fact  which  supports  the  view  that  the 
cortical  area  concerned  with  olfactory  sensations  is  that 
of  the  uncinate  gyrus  or  its  immediate  neighbourhood. 

One  such  case  which  the  author  had  an  opportunity  of 
observing  was  that  of  a  young  man  under  the  care  of 
Sir  James  Kingston  Fowler  *  at  the  Middlesex  Hospital. 
Along  with  general  signs  of  cerebral  abscess  there  were 
frequent  attacks  of  epileptic  vertigo,  which  were  pre- 
ceded by  a  sense  of  an  unpleasant  smell.  After  death 
a  large  abscess  was  found  in  the  fore  part  of  the  temporo- 
sphenoidal  lobe. 

Another  important  fact  connected  with  this  case  was 
the  excessive  hunger  and  thirst  from  which  the  patient 
suffered,  a  condition  which  is  occasionally  found  in 
connection  with  injuries  and  diseases  of  various  parts 
of  the  brain,  but  more  especially,  it  would  appear,  when 
the  lesion  is  in  the  locality  of  the  temporo-sphenoidal 
lobe,  a  point  which  has  been  brought  out  by  Stephen 
Paget  t  from  a  survey  of  14  cases  of  the  kind  which  he 
collected; 

*  8ee  "Post-mortem  Eecords  of  the  Middlesex  HosiDital,"  1895. 
t  Trans,  of  the  Clin.  Soc.  of  London,  1897. 


366    DISEASES   OF   THE   NERVOUS   SYSTEM 

Thus,  sensations  of  smell,  together  with  "  dreamy 
mental  states,""^  and  perhaps  voracious  hunger  and  thirst, 
are  the  signs  which  suggest  that  a  tumour  is  situated  in 
the  neighbourhood  of  the  uncinate  convolution. 

3.  Tumours  of  the  optic  thalamus. — It  is  often 
very  difficult  to  locate  tumours  of  the  optic  thalamus. 
Most  of  the  symptoms  are  due  to  pressure  on  the 
internal  capsule,  and  therefore  consist  of  hemiplegia, 
hemianaesthesia,  and  hemianopia.  Loss  of  control  over 
the  emotional  movements,  the  early  appearance  of 
hemiansesthesia  and  athetoid  movements,  are  some  of 
the  signs  that  are  most  likely  to  occur. 

4.  Tumours  of  the  corpora  quadrigemina. — 
In  tumours  of  this  region  there  is  generally  well- 
marked  paralysis  of  the  eye  muscles,  probably  partly 
produced  by  pressure  on  the  nuclei  of  the  3rd  nerves. 
The  reaction  of  the  pupils  both  to  light  and  to 
accommodation  is  often  impaired,  and  nystagmus  may 
occasionally  be  observed.  There  is  also  difficulty  in 
maintaining  the  equilibrium  and  a  tendency  to  fall 
in  one  direction. 

5.  Tumours  of  the  corpus  caJIosum. — It  is 
but  seldom  that  a  growth  beginning  in  the  corpus 
callosum  can  be  localized  with  any  degree  of  certainty. 
As  a  rule,  the  general  symptoms  develop  slowly,  and 
no  localizing  signs  occur  until  the  tumour  has  invaded 
one  or  other  hemisphere  sufficiently  far  to  injure  the 
motor  fibres.  In  some  cases  mental  changes  have  been 
observed,  suggesting  a  lesion  of  the  frontal  lobes. 

6.  Tumours  at  the  base  of  the  brain. — Tumours 
at  the  base  of  the  brain  are  generally  accompanied  by 
early  signs  of  palsy  of  cranial  nerves,  the  situation  of 
which  is  depicted  in  Plate  19. 

i.  In  tumours  of  the  pituitary  body  the  optic  chiasma 
is  very  apt  to  suffer,  and  if  it  be  compressed  at  its 
centre  a  double  temporal  hemianopia  will  ensue,  followed 
by  mOiO  complete  loss  of  vision  as  the  pressure  extends 
to  the  fibres  which  constitute  the  temporal  portions  of 
the  optic  nerves  [see  Fig.  17,  p.  85).     These  symptoms 


PLATE  19.— The  cranial  nerves  at  the  base  of  the  brain. 

{Frorn  a  painting  hij  Sir  Charles  Bell  at  the  Middlesex  Hospital 
Medical  School.) 


TUMOUES   AT   BASE   OF   BRAIN  367 

are  often  the  first  complained  of  by  patients  suffering 
from  acromegaly,  the  characters  of  which  are  described 
at  p.  378. 

Tumours  growing  in  the  middle  fossa  generally  para- 
lyse the  ocular  and  trigeminal  nerves,,  while  those  of 
the  posterior  fossa  affect  the  6th  nerves  very  early,  and 
signs  of  pressure  on  the  crus,  pons,  and  cerebellum  may 
appear. 

ii.  Tumours  at  the  angle  of  the  pons  and  cerebellum. — 
Tumours  growing  at  the  angle  of  the  pons  and  cere- 
bellum are  of  special  interest  from  the  relative  frequency 
of  their  occurrence  and  the  possibility  of  their  removal. 
Although  this  region  of  the  brain  is  not  exempt  from 
tumours  of  every  kind,  the  one  that  is  especially  prone  to 
arise  is  of  a  nodulated  fibrous  appearance,  and  not  in- 
frequently attains  the  size  of  a  walnut  or  a  pigeon's  egg. 

These  growths  have  been  variously  described  as 
fibro-neuromata  and  sarcomata,  and  often  appear  to 
arise  from  the  sheath  of  the  auditory  nerves.  Bland- 
Sutton  looks  upon  some  of  them  as  psammomata,  and 
believes  that  they  have  originated  from  processes  of  the 
chorionic  villi  of  the  fourth  ventricle.  Sometimes  they 
are  bilateral. 

These  tumours  produce  localizing  symptoms  from 
pressure — 

(1)  On    the    cranial    nerves    in    their    immediate 

vicinity. 

(2)  On  the  cerebellum. 

(3)  On  the  pons  and  medulla. 

Eeference  to  Plate  20  will  make  these  three  lines 
of  pressure  clear. 

(1)  Pressure  on  the  cranial  nerves. — The  nerves  most 
likely  to  suffer  are  the  5th,  6thj  7th,  and  8th,  and,  as  the 
tumour  generally  arises  in  close  apposition  to  the  auditory 
nerve,  deafness  is  often  one  of  the  earUest  symptoms. 

The  facial  trunh  is  generally  compressed  at  the  same 
time,  but  it  is  apparently  more  capable  of  resisting 
pressure  than  the  auditory  nerve,  so  that  the  facial 
muscles  often  suffer  to  a  surprisingly  slight  extent. 


368    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  QtTi  nerve  is  affected  early  through  displacemePxt 
of  the  parts,  and  weakness  of  the  external  rectus  with 
its  corresponding  symptoms  results. 

The  bth  nerve  becomes  compressed  as  the  tumour 
extends  forward,  and,  in  consequence,  pain  is  complained 
of  over  some  part  of  the  area  of  its  distribution. 

(2)  Pressure  on  the  cerebellum. — As  the  tumour 
presses  backwards  on  to  one  of  the  lateral  lobes  of 
the  cerebellum  it  gives  rise  to  all  the  symptoms  of  a 
tumour  primarily  situated  in  that  position,  viz. :  nystag- 
mus, ataxy,  lurching  in  one  or  other  direction,  weakness 
from  loss  of  muscle  tone  of  the  arm  and  leg  on  the 
same  side,  and  frequent  attacks  of  giddiness — symptoms 
which  are  also  discussed  under  the  heading  of  Cere- 
bellar Tumours. 

(3)  Pressure  on  the  pons  and  medulla. — The  pons  and 
medulla  are  often  greatly  displaced  towards  the  opposite 
side — a  point  which  is  well  shown  in  the  accompanying 
illustration  (Plate  20). 

The  displacement  tends  to  cause  a  weakness  of  the 
arm  and  leg  of  ordinary  hemiplegic  character  on  the 
opposite  side,  since  the  motor  fibres  to  the  limbs  are  com- 
pressed a  little  above  the  point  at  which  they  decussate. 
The  face  on  the  opposite  side  to  the  tumour  will  seldom 
be  affected  (the  probability  of  some  facial  paralysis 
on  the  same  side  as  the  tumour  has  already  been  men- 
tioned), since  the  fibres  to  the  nucleus  of  the  7th  nerve 
have  already  crossed  and  are  usually  beyond  reach  of 
the  tumour.  Considering  the  degree  of  displacement 
that  often  occurs,  one  would  expect  the  effects  to  be 
very  great,  but  they  are,  in  fact,  often  but  slight, 
for,  the  growth  being  slow,  the  fibres  are  so  gradu- 
ally compressed  that  they  are  able  to  adjust  them- 
selves to  the  altered  conditions  with  very  little  loss  of 
function. 

In  the  case  illustrated  there  was  no  definite  hemi- 
plegia on  the  side  opposite  to  the  tumour,  and  it  affords 
an  excellent  example  of  the  power  of  preservation  of 
function  which  nerve  fibres  possess  when  the  pressure  on 


?;ft-wf\- 


^^,' 


PLATE  20.— Tumour  situated  at  the  angle  of  the  pons  and 
cerebellum. 


TUMOURS   AT   BASE    OF   BRAIN  369 

them  is  only  very  slowly  increased.  The  main  points 
to  be  borne  in  mind  for  localizing  tumours  in  this 
situation  are — 

(a)  Pressure  on  the  auditory  (chiefly),  facial,  abdu- 
cens  (6th),  and  trigeminal  (5th)  nerves  on  the  name 
side  as  the  tumour, 

(b)  Symptoms  of  disease  of  the  lateral  lobe  of  the 
cerebellum,  also  on  the  smne  side. 

(c)  Weakness  of  arm  and  leg  with  increased  deep 
reflexes  and  extensor  type  of  plantar  reflex  on  the  opposite 
side. 

Since  these  tumours  usually  press  upon  three  different 
sets  of  structures,  namely,  cranial  nerves,  cerebellum, 
pons  and  medulla,  it  is  obvious  that  difficulty  must 
sometimes  be  experienced  in  deciding  which  of  the  three 
parts  is  the  primary  site  of  the  growth.  In  other  words, 
is  the  growth  situated  in  the  angle  between  the  pons  and 
cerebellum,  in  the  cerebellum,  or  in  the  pons  ? 

Early  signs  of  implication  of  the  cranial  nerves — 
as,  for  instance,  when  deafness,  along  with  weakness 
of  the  facial  muscles  and  noises  in  the  ear,  makes  its 
appearance  slowly  and  gradually,  before  anything  else 
is  noticed — are  strong  evidence  that  the  tumour  is  in 
the  pontine-cerebellar  angle.  In  cases  of  primary  intra- 
cerebellar  growth,  these  cranial  nerves,  with  the  excep- 
tion of  the  6th,  would  not  be  likely  to  suffer  till  other 
symptoms  were  well  developed.  Grainger  Stewart  and 
Gordon  Holmes  have  observed  that,  in  the  vertigo 
accompanying  tumour  of  the  lateral  lobe  of  the  cerebel- 
lum, the  direction  in  which  the  patient  feels  himself 
to  be  rotating  is  away  from  the  side  of  the  lesion  ;  while 
in  cases  of  extracerebellar  tumour  the  sensation  of 
turning  is  towards  the  side  of  the  lesion.*  The  chief 
effects  of  tumours  of  the  pons  have  already  been  con- 
sidered, and  the  prominence  of  "  crossed  paralysis," 
conjugate  deviation  of  the  eyes,  and  paresis  (sometimes 
bilateral)  will  generally  serve  to  distinguish  pontine 
tumours  from  tho3  3  now  under  consideration. 
*  Brain,  Part  cviii, 

y 


570    DISEASES   OF  THE  NEEVOUS   SYSTEM 

7.  Tumours  of  the  cerebellum. — In  tumours  of 
the  cerebellum  the  general  symptoms  of  an  intracranial 
growth  are,  as  a  rule,  exceptionally  well  marked. 

Optic  neuritis  is  more  frequently  met  wath  in  associa- 
tion with  these  tumours  than  with  those  of  any  other 
part  of  the  brain.  It  comes  on  early,  and  the  inflam- 
matory process  is  apt  to  be  more  advanced  in  the  eye 
of  the  same  side  as  the  growth.  Headache  is  usually 
very  intense,  and  often  referred  to  one  or  other  side  of 
the  occipital  region ;  there  may  also  be  rigidity  of  the 
neck  muscles.  Vertigo  and  vomiting  are  likewise  generally 
prominent  symptoms  and  appear  at  an  early  date. 

The  influence  of  each  half  of  the  cerebellum  is 
chiefly  exerted  on  the  same  side  of  the  body,  in  con- 
tradistinction to  that  of  the  cerebrum,  where  each 
half  mainly  governs  the  opposite  side  ;  tumours  and 
other  diseases  of  the  cerebellum  therefore  tend  to  pro- 
duce symptoms  on  the  same  side  of  the  body  as  the 
lesion. 

The  signs  of  a  cerebellar  tumour  may  be  divided  into 
two  groups,  according  as  they  depend  directly  upon  the 
disordered  function  of  the  cerebellum,  or  indirectly  upon 
pressure  on  neighbouring  parts. 

(1)  Direct  effects  of  cerebellar  lesions.  Motor  symptoms. — 
These  are  generally  weakness  and  want  of  tone  in  the 
limbs  on  the  same  side  as  the  tumour.  This  loss  of 
power,  often  slight,  on  the  same  side  as  the  lesion,  and 
due  directly  to  deficiency  of  cerebellar  function,  must 
not  be  confused  with  paresis  on  the  opposite  side,  which 
may  arise  from  tie  effects  of  pressure  on  the  medulla  or 
pons. 

Incoordination,  usually  more  prominent  on  the 
si  le  of  the  lesion,  is  especially  marked  in  the  lower 
limbs,  but  is  not  usually  increased  when  the  eyes  are 
closed.  One  effect  of  incoordination  of  the  upper  limbs 
which  is  sometimes  seen  is  an  inability  to  pronate  and 
supinate  the  forearm  rapidly. 

The  gait  is  of  a  reeling  character,  and  the  legs  are 
kept  wide  apart ;    there  is  also  a  tendency  to  turn  and 


CEREBELLAR   TUMOURS  371 

fall  to  one  side,  usually,  though  not  always,  towards 
the  side  of  the  tumour. 

Compensatory  attitudes  are  sometimes  assumed  in 
attempting  to  neutralize  the  tendency  to  incline  to- 
wards the  diseased  side.  These  are  often  excessive, 
and  cause  the  patient  to  incline  unduly  towards  the 
sound  side. 

Tremors  are  often  present. 

Tonic  spasms,  causing  opisthotonos  and  rigidity 
of  the  whole  body,  have  sometimes  been  noted,  but  they 
are  uncommon.  Occasionally  there  are  jerky  move- 
ments, and,  unless  care  be  taken  to  inquire  closely 
into  the  character  of  such  attacks,  a  patient's  descrip- 
tion of  the  condition  may  suggest  some  similarity  to  a 
Jacksonian  fit. 

Position  of  head. — It  has  been  shown  that  in  a 
certain  number  of  cases  the  head  is  tilted  so  that  the  ear 
approximates  to  the  shoulder  on  the  side  opposite  the 
lesion,  while  the  face  is  turned  up  to  the  side  of  the 
lesion. 

Forced  movements,  in  which  the  patient  rotates  round 
the  long  axis  of  his  body,  sometimes  occur. 

Ocular  movements. —  Nystagmus  is  very  frequently 
present.  Sometimes  it  is  lateral,  and  brought  out  by 
fixing  the  eyes  to  one  or  other  side  ;  at  other  times  it  is 
associated  with  all  the  movements  of  the  eyeball,  and  it 
may  be  of  a  rotary  kind.  The  movements  are  often 
of  different  character,  according  to  whether  the  eyes 
are  turned  to  the  right  or  left,  being  short  and  sharp 
in  the  one  instance,  and  longer  and  slower  in  the 
other.  In  such  cases  the  more  deliberate  movements 
generally  occur  on  the  same  side  as  the  tumour. 

Skew  deviation,  where  one  eye  is  turned  downwards 
and  inwards  and  the  other  upwards  and  outwards,  is 
sometimes  observed.  In  a  case  of  cerebellar  abscess 
under  the  care  of  the  author,  both  eyes  were  turned 
downwards  and  inwards. 

Weakness  of  the  external  rectus,  which  is  so  frequent 
in  cerebellar  disease,  is  due  to  pressure  on  the  6th  nerve. 


372      DISEASES   OF   THE   NERVOUS   SYSTEM 

and  reference  is  made  to  this  in  connection  with  the 
indirect  results  of  these  tumours  {see  below). 

Vertigo  is  generally  a  prominent  symptom  and  occurs 
in  paroxysms. 

Reflexes. — The  knee-jerks  vary  in  different  cases  ; 
if  they  are  unequal,  the  more  active,  according  to  experi- 
mental results,  is  likely  to  be  on  the  side  of  the  lesicn 
(Risien  Russell). 

In  tumours  of  the  middle  lobe  there  may  be  retraction 
of  the  head,  arching  of  the  back,  and  extension  of  the 
legs  (cerebellar  attitude  of  Hughlings  Jackson),  which 
position  may  be  further  intensified  by  the  occurrence 
of  tetanus-like  seizures.  There  is  generally  a  tendency 
to  fall  backwards. 

'2)  Indirect  effects  of  cerebellar  lesions.  —  Of  the 
symjDtoms  produced  by  pressure  on  the  jjarts  around, 
the  most  frequent  is  a  paralysis  of  the  6th  nerve  on 
the  same  side  as  the  lesion,  which  leads  to  weakness  of 
the  external  rectus  and  subsequently  to  a  tendency 
for  the  eyes  to  deviate  from  the  affected  side.  Other 
cranial  nerves,  such  as  the  auditory,  facial,  and  tri- 
geminal, may  be  injured,  though,  as  a  rule,  much  less 
severely  in  cerebellar  tumours  than  in  those  which  arise 
at  the  angle  of  the  cerebellum  and  pons. 

Pressure  on  the  pons  may  cause  crossed  paralysis,  i.e. 
hemiplegic  symptoms  on  the  opposite  side  of  the  body, 
with  ocular  paralysis  on  the  same  side  ;  while  pressure 
on  the  medulla  may  produce  pharyngeal  and  laryngeal 
symptoms.  Attacks  of  dyspnoea  are  also  apt  to  occur 
from  pressure  on  the  medulla,  and  may  be  a  cause  of 
sudden  death. 

Wlien  the  tumour  grows  slowly  it  may  cause  hydro- 
cephalus, the  symptoms  of  which  may  mask  those  pro- 
duced directly  by  the  primary  disease. 

Diagnosis  of  intracranial  tumours. — The  diag- 
nosis of  intracranial  tumour  is  made  from  the  general 
signs  of  a  growth,  aided  by  the  local  ones  if  present. 

Mistakes  are  most  likely  to  arise  when  headache  and 
vomiting  and  perhaps  optic  neuritis  are  present  from 


INTKACRANIAL   TUMOURS:    PROGNOSIS    373 

other  causes,  such  as  severe  anwmia,  chronic  lead  foi" 
soning,  and  especially  Brighfs  disease  with  albuminuric 
retinitis  {see  Plate  5,  Fig.  3,  facing  p.  82).  Where  the 
symptoms  are  very  obscure  a  case  may  even  for  a  time 
be  regarded  as  functional. 

Having  decided  that  an  intracranial  tumour  is 
present,  it  is  necessary  to  try  (1)  to  locate  its  position, 
(2)  to  infer  its  probable  structure,  and  (3)  to  decide 
whether  any  attempt  shall  be  made  to  remove  it. 

Prognosis  and  treatment. — The  prognosis  must 
depend  upon  the  conclusions  that  are  arrived  at  con- 
cerning (1)  the  nature  of  the  tumour,  (2)  the  probability 
of  its  removal  by  medical  or  operative  measures,  and  (3) 
the  possibility  of  the  occurrence  of  spontaneous  retro- 
gression. A  benign  tumour  is,  other  things  being  equal, 
obviously  more  hopeful  than  one  that  is  malignant,  and 
should  the  latter  be  a  secondary  deposit  the  outlook 
will  in  most  cases  naturally  be  hopeless.  It  is  in  syphilitic 
growths  that  most  is  to  be  expected  from  medicinal 
measures,  and  when  there  is  the  least  chance  of  the 
disease  being  of  this  nature  a  thorough  com'se  of 
treatment  should  be  given.  Tuberculous  deposits  often 
show  a  tendency  to  retrogress  under  the  influence  of 
cod-liver  oil,  iron,  good  food,  and  fresh  air. 

With  these  exceptions  no  rehance  can  be  placed 
on  drugs,  and,  if  no  benefit  be  obtained  from  a  thorough 
but  brief  trial  of  treatment,  the  desirability  of  an 
attempt  to  remove  the  tumour  must  be  seriously  con- 
sidered. The  decision  must  depend  chiefly  on  the 
position  and  apparent  accessibility  of  the  growth  and 
on  the  probable  effects  of  its  attempted  removal. 

Tumours  of  the  frontal,  parietal,  occipital,  and 
temporo-sphenoidal  cortex  can  all  be  reached,  as  also 
can  those  of  the  cerebellum  and  cerebello-pontine  angle. 

While  the  danger  from  an  operation  is  in  all  cases 
considerable,  it  diiiers  according  to  the  position  of  the 
tumour,  being  less  when  the  growth  is  situated  in  the 
mid-  and  fore -part  of  the  brain,  and  greater  when  it  is  in 
the  region  of  the  cerebellum.     Thus,  although  a  tumour 


374:    DISEASES   OF  THE  NERVOUS   SYSTEM 

of  the  lateral  recess  (cerebello-pontine  angle)  may  be 
easily  shelled  out  of  its  bed,  the  shock  produced  in 
reaching  it  is  great  enough  to  make  the  mortality  a  very 
high  one. 

Where  removal  of  the  tumour,  owing  to  its  size, 
nature,  or  position,  cannot  be  accomplished,  an  en- 
deavour should  be  made  to  relieve  the  symptoms  and 
prolong  life  by  the  operation  of  decompression,  i.e.  by 
removal  of  a  large  piece  of  bone  and  (usually)  incision 
of  the  dura  mater.  By  this  means  the  intracranial 
pressure  is  lowered,  the  beneficial  result  of  which  is 
especially  seen  in  the  consequent  tendency  of  the  optic 
neuritis  to  subside — a  matter  of  the  greatest  import- 
ance in  those  cases  where  the  growth  of  the  tumour 
eventually  becomes  stationary.  Apart,  therefore,  from 
other  considerations,  the  operation  of  decompression  is 
a  valuable  means  of  preserving  the  sight.  Care  must  be 
taken  not  to  allow  the  changes  in  the  retina  to  proceed 
too  far  before  something  is  done,  and,  during  the  period 
that  medicinal  treatment  is  being  carried  out,  careful 
observations  must  frequently  be  made  on  the  condition 
of  the  optic  discs  and  on  the  fields  of  vision,  for  any 
rapid  contraction  of  the  latter  indicates  the  necessity 
of  relieving  the  pressure  without  further  delay. 

The  smaller  degree  of  shock  w^hich  follows  the  opera- 
tion of  decompression  makes  it  a  far  less  dangerous 
procedure  than  that  of  removal  or  attempted  removal 
of  the  tumour,  and,  considering  the  high  mortality  and 
the  disappointing  results  that  follow  the  latter  operation, 
it  is  well  to  be  content  with  decompression  unless  the 
circumstances  are  unusually  favourable  to  the  total 
removal  of  the  growth. 


CHAPTER  XXXVIII 

DISEASES    OF    THE    PITUITARY    BODY 

The  pituitary  gland  is  situated  in  the  sella  turcica 
of  the  sphenoid  bone,  and  consists  of  an  anterior  and 
a  posterior  lobe.     (Fig.  95.) 


Fig.  95.— Base  of  brain  showing  position  of  pitiiitary  body 
just  behind  the  optic  commissure. 

The  anterior  lobe  arises  as  an  outgrowth  of  the 
pharynx,  and  is  composed  of  glandular  tissue  which 
bears  considerable  similarity  in  structure  to  that  of  the 

375 


376    DISEASES   OF   THE   NERVOUS   SYSTEM 

thyroid  bcdy.  The  posterior  lobe  arises  from  the  mid- 
brain, to  which  it  remains  attached  by  a  stalk  known 
as  the  infundibulum,  and  is  composed  chiefly  of  loosely 
arranged  neuroglial  tissue,  together  with  pigmented  cells 
and  blood-vessels.  Between  these  two  lobes  there  is  a 
layer  known  as  the  pars  intermedia,  the  structure  of 
which  is  partly  glandular  and  partly  vascular. 

The  secretion  of  the  posterior  lobe  exerts  an  influ- 
ence to  some  extent  similar  to  that  of  the  secretion 
of  the  suprarenal  body,  inasmuch  as  it  causes  a  rise  of 
blood-pressure,  but  its  chief  function  seems  to  be  con- 
cerned with  carbohydrate  metabolism.  Over-activity  of 
this  lobe  is  apt  to  be  associated  with  glycosuria,  while 
diminished  secretion  is  accompanied  by  a  tolerance  of 
carbohydrates  and  a  consequent  tendency  to  obesity. 

Cushing's  experiments  on  adult  dogs  show  that  when 
the  pituitary  gland  is  removed,  with  the  exception  of 
a  fragment  of  the  anterior  lobe,  the  resulting  changes 
are  adiposity,  increased  toleration  of  sugar,  subnormal 
temperature,  and  reversive  sexual  changes,  while  in 
puppies  retarded  skeletal  growth,  psychic  disorder,  and 
sexual  infantilism  occur. 

Cases  are  met  with  clinically  in  which  symptoms 
similar  to  these  are  present.  They  are  generally  classed 
under  the  headings  of  Frohch's  disease,  or  dystrophia 
adiposo-genitahs  (Fig.  96),  and  are  characterized  by 
adiposity  with  skeletal  and  sexual  infantiflsm.  In  such 
instances  it  may  usually  be  inferred  that  the  posterior 
lobe  of  the  pituitary  body  is  at  fault,  but  when  making 
that  diagnosis  it  must  not  be  forgotten  that  abnor- 
malities of  the  suprarenal  and  the  sexual  glands,  with 
which  the  function  of  the  pituitary  is  closely  correlated, 
may  also  be  partly  responsible  for  the  condition. 

The  anterior  lobe  of  the  pituitary  bcdy  is  closely 
concerned  with  skeletal  growth,  and  when  its  function 
is  disturbed  on  the  side  of  excessive  secretion  (hyper- 
pituitarism) an  overgrowth  of  the  skeletal  structures 
takes  place  which,  according  to  circumstances,  results 
in  the  production  of  giantism  or  of  acromegaly. 


PITUITAKY   DISEASES 


377 


If  the  disease  occurs  while  the  subject  is  still  young 
n  nd  before  ossij&cation  of  the  epiphyses  has  taken  place, 


Fig.  96.— Dystrophia  adiposo-genitalis  in  a  boy  aged  11, 
(Case  under  the  care  of  Dr.  H.  G.  Turney.) 

the  result  is  giantism,  while  in  older  people  the  growth 
takes  place  in  other  directions,  with  the  resulting  disease 


378    DISEASES   OF  THE  NERVOUS   SYSTEM 

known  as  acromegaly.  There  is  thus  a  possibility  of 
the  symptoms  beginning  with  those  of  giantism  while 
the  bones  are  capable  of  growing  longer,  and  ending 
with  those  of  acromegaly  when  age  has  rendered  an 
increase  in  length  no  longer  possible. 

The  classification  of  cases  of  pituitary  disease  into 
states  of  over-activity  (hyperpituitarism)  and  under- 
activity (hypopituitarism)  must  not  be  taken  too  rigidly, 
for  Gushing  has  shown  that  it  is  common  to  find  a 
phase  of  over -secretion  followed  later  by  one  in  which 
secretion  is  diminished,  and  in  such  a  case  symptoms 
of  the  later  stage  of  deficiency  may  be  added  to  those 
of  the  earlier  one  of  over-activity.  Hence  Gushing 
considers  that  in  the  majority  of  cases  the  term 
"  dyspituitai'ism  "  is  the  more  appropriate,  and  he 
suggests  a  classification  as  follows  : 

1.  Gases  in  which  symptoms  of  hyperpituitarism 

predominate,  producing  either  giantism  or 
acromegaly,  according  to  the  age  at  which 
the  patient  is  affected. 

2.  Cases  in  which  the   symptoms   of  hypopitui- 

tarism predominate,  with  the  consequent 
presence  of  adiposity  and  sexual  infantilism. 

3.  Gases  in  which  the  symptoms  exhibit  features 

of  both  states. 

Acromegaly 

Acromegaly  w^as  first  fully  described  by  Pierre 
Marie,  who  associated  the  condition  with  enlargement  of 
the  pituitary  gland.  Subsequent  observations  showed 
that  enlargement  does  not  always  occur,  and  for  a  time 
there  was  considerable  doubt  as  to  the  relationship  of 
the  gland  to  the  symptoms,  until  it  became  recognized 
that  abnormalities  in  the  quantity  and  probably  also 
in  the  quality  of  the  secretion  can  occur  without  enlarge- 
ment of  the  gland  necessarily  taking  place. 

Symptoms  and  morbid  anatomy. — The  symp- 
toms of  acromegaly  may  be  divided  into  two  groups  : 


ACKOMEGALY  379 

(1)  those  directly  due   to    enlargement    of    the  gland, 

(2)  those  due  to  changes  in  general  metabohsm. 

One  of  the  most  important  local  results  of  an  enlarged 
pituitary  body  is  a  disturbance  of  vision,  generally 
attributed  to  pressure  on  the  optic  chiasma.  The  most 
striking  form  which  this  disturbance  takes  is  the  de- 
velopment of  bitemporal  hemianopia,  followed  later 
by  extension  of  the  blindness  into  the  nasal  fields ;  and 
it  is  interesting  to  note  that  in  these  cases  perimetric 
observations  show  further  differentiation  in  that  a  piece 
of  the  field  in  the  upper  and  outer  part  of  the  temporal 
half  is  generally  obliterated  before  the  rest. 

The  explanation  usually  given  for  this  sequence 
of  events  is  an  anatomical  one,  which  depends  upon 
the  arrangement  of  the  optic  fibres  at  the  chiasma. 
Reference  to  Fig.  17  (p.  85)  shows  that  the  fibres 
from  the  inner  half  of  the  retina  which  correspond  to 
the  temporal  field  of  vision  decussate  with  their  fellows 
of  the  opposite  side,  while  those  from  the  outer  part 
of  the  retina  which  correspond  to  the  nasal  fields  of 
vision  pass  backwards  from  the  outer  side  of  the  retina 
without  crossing.  Thus  any  pressure  on  the  centre  of 
the  chiasma  such  as  might  be  expected  to  be  exerted 
by  an  enlarging  pituitary  gland  would  first  of  all  tend 
to  press  upon  the  crossing  fibres  and  produce  bitemporal 
hemianopia,  while  later  on  it  would  interfere  with  the 
outer  fibres  and  so  affect  the  nasal  fields.  On  the  other 
hand,  when  the  pressure  was  unequal  from  the  beginning, 
the  failure  of  the  fields  would  not  show  any  definite 
division  into  temporal  and  nasal  halves.  The  difficulty 
of  accepting  this  explanation  in  its  entirety  lies  in  the 
fact  that  the  size  of  the  whole  chiasma  is  so  small  that 
it  is  not  easy  to  see  how  pressure  can  be  limited  to  the 
crossing  fibres  for  any  length  of  time.  It  may  be  that, 
given  equal  pressure  on  all  the  fibres,  the  function  of  some 
fails  before  that  of  others.  There  are  examples  of  this  in 
other  nervous  structures,  such  as,  for  instance,  in  the  case 
of  the  recurrent  laryngeal  nerve  as  described  at  p.  110; 
However  this  may  be,  a  temporal  hemianopia,  followed 


380    DISEASES   OF   THE  NERVOUS  SYSTEM 

later  by  pallor  of  the  discs,  is  a  very  suggestive  symptom 
of  pituitary  pressure,  and,  in  the  absence  of  any  indica- 
tion to  the  contrary,  it  should,  at  any  rate  provisionally, 
generally  be  regarded  in  this  light. 

This  sequence  of  events  as  regards  loss  of  vision  is, 
however,  by  no  means  constant,  for  it  frequently  happens 
that  both  visual  fields  contract  without  any  preliminary 
discrimination  for  the  temporal  half  being  observed, 
though  it  is  not  often  that  both  eyes  are  equally  affected 
until,  at  any  rate,  the  later  stages  in  the  disease  have 
been  reached.  In  some  instances  homonymous  hemi- 
anopia  may  be  the  first  change,  and  in  others  a  central 
scotoma  expanding  into  the  temporal  field  may  be  a 
notable  feature  of  the  failing  sight.  Failure  of  vision 
for  colours  has  been  observed  to  occur  before  that  for 
form,  and  according  to  Gushing  this  order  of  events  is 
the  rule. 

Herbert  Fisher  considers  that  the  visual  symptoms 
in  many  cases  are  best  explained  by  the  occurrence  of 
traction  on  the  visual  pathways,  as  the  tumour  extends 
upwards  behind  the  chiasma  and  between  the  optic  tracts 
in  the  interpeduncular  space.  He  thinks  that  a  tumour 
which  is  fairly  symmetrical  in  outline  might  stretch  the 
decussating  fibres  in  the  chiasma  without  dragging  on 
the  direct  fibres,  and  so  cause  a  bitemporal  hemianopia. 
On  the  same  hypothesis  can  be  explained  the  cases  of 
expanding  scotoma,  and  hemiachromatopsia  can  also 
be  more  easily  accounted  for  than  on  the  assumption 
of  the  direct -pressure  causation.  There  is  evidence, 
moreover,  to  show  that  the  loss  of  function  of  vision  is 
often  in  advance  of  the  apparent  structural  changes ; 
and  from  the  recovery  of  vision  which  has  resulted 
from  relief  of  pressure,  Gushing  concludes  that  "  it 
is  safe  to  say  that  the  amblyopia  associated  with  a 
primary  atrophy  more  often  represents  a  physiological 
block  to  light  impulses  than  an  actual  destruction  of 
the  nerves." 

Headache  in  pituitary  tumours  is  due  to  rise  of 
intracranial  pressure.     It  is,  however,  important  to  note 


ACROMEGALY  381 

that  the  headache  is  not  necessarily  relieved  by  the 
ordinary  decompression  operation,  because  the  gland 
is  closed  in  by  dura  mater  in  such  a  way  as  to  form  a 
local  sac,  which  must  itself  be  tapped  if  adequate  relief 
is  to  be  obtained.  Mucous  discharge  into  the  pharynx 
may  occur  as  the  result  of  changes  in  the  neighbouring 
sphenoidal  sinuses.  Enlargement  and  alteration  in  shape 
of  the  sella  turcica  often  occurs,  and  when,  as  frequently 
happens,  it  can  be  detected  by  means  of  the  X-rays, 
it  becomes  a  fact  of  great  diagnostic  importance. 

The  changes  due  to  alteration  in  general  metabolism 
show  themselves  chiefly  in  overgrowth  of  the  skeletal 
tissues. 

In  the  head  (Fig.  97),  enlargement  of  the  upper  and 
lower  jaws  is  among  the  most  prominent  characteristics, 
and  the  face  assumes  a  heavy  and  elongated  aspect. 
At  the  same  time  the  features  become  broadened  and 
coarsened  owing  to  changes  in  the  soft  tissues  and  car- 
tilages. The  bony  sinuses  become  dilated,  thus  causing 
undue  prominence  of  the  cheeks  and  of  the  orbits,  and 
giving  a  peculiar,  massive  expression  to  the  countenance. 
The  nose  becomes  thickened,  chiefly  due  to  the  increase 
in  thickness  of  its  cartilage,  and  the  cartilages  of  the 
ear  are  often  subject  to  changes  of  a  similar  character. 
A  change  in  the  bones  which  almost  invariably  takes 
place  sooner  or  later  is  curvature  of  the  spine  (Fig.  98), 
and  indeed  this  symptom  ranks  among  those  which  are 
most  typical  of  the  disease.  The  vertebrae  enlarge  and 
form  an  antero-posterior  as  well  as  a  lateral  curve. 
Of  the  two,  the  antero-posterior  curve  is  usually  the 
greater,  and  causes  the  rounded  shoulders  and  the 
hanging  head — which  can  only  with  difficulty  be  held 
up  straight.  The  bones  of  the  thoi^ax  are  also  altered. 
The  hands  and  feet  are  early  and  greatly  enlarged, 
and  it  was  from  this  fact  that  Marie  called  the  con- 
dition acromegaly. 

The  changes  are  not  by  any  means  limited  to  the 
bones  ;  the  subcutaneous  tissue  becomes  infiltrated  in 
a  manner  somewhat  similar  to  the  infiltration  which 


382    DISEASES  OF  THE  NERVOUS  SYSTEM 

occurs  in  myxoedema,  with  the  result  that  the  parts 
become  swollen  and  puffy.  In  the  face  this  swelling 
and  puffiness  add  to  the  coarsening  of  the  features,  which 


Fig.  97.— A  typical  case  of  acromegaly. 

are  already  broadened  by  the  changes  in  the  bones  and 
cartilages.  This  puffiness  is  very  noticeable  in  the  lips, 
and  more  especially  in  the  lower  lip,  which  assumes  a 


ACEOMEGALY  383 

thickened,  pouting,  overhanging  appearance.  The  tongue 
is  increased  in  size  and  becomes  too  large  to  fit  comfort- 
ably in  the  mouth.     The  hands  are  broadened,  with  the 


Pig.  98.— Side  view  of  same  patient  as  in  Fig.  97,  showing 
the  curvature  of  the  spine. 

ends  of  the  fingers  blunted,  and  have  been  compared  in 
their  shape  to  a  spade  or  a  battledore.  The  feet  under- 
go changes  of  a  similar  nature. 


384     DISEASES   OF   THE   NERVOUS  SYSTEM 

Alterations  in  the  size  of  the  thyroid  have  frequently 
been  noted,  and  as  a  general  rule  the  change  is  in  the 
direction  of  diminution.  An  area  of  dullness  over  the 
upper  portion  of  the  sternum  has  sometimes  been  ob- 
served, and  has  been  attributed  to  an  increase  in  the 
size  of  the  thymus  gland,  while  the  occasional  presence 
of  sugar  in  the  urine  is  suggestive  of  a  disturbance  of 
the  secreting  properties  of  the  pancreas. 

Transient  symptoms  of  acromegaly  have  been 
occasionally  observed  to  occur  during  pregnancy,  due 
presumably  to  changes  in  the  pituitary  gland  analogous 
to  those  which  sometimes  occur  in  the  thyroid  during 
this  state. 

Treatment. — Medicinal  treatment  is  practically 
limited  to  the  use  of  preparations  of  the  thyroid  or 
pituitary  gland. 

The  benefit  obtained  from  pituitary  extracts  is 
variable,  but  they  are  indicated  when  there  is  reason 
to  believe  the  secretion  of  the  gland  is  diminished. 

Thyroid  preparations  undoubtedly  do  good  in  some 
cases,  both  by  improving  the  sight  and  by  checking  the 
progress  of  the  more  general  symptoms  of  the  disease. 

The  explanation  of  the  beneficial  action  of  thyroid 
is  to  be  found  in  the  close  relationship  that  exists 
between  the  functions  of  this  gland  and  those  of  the 
pituitary  body. 

It  has  been  shown  experimentally  that  the  removal 
of  the  thyroid  in  animals  may  be  followed  by  an  enlarge- 
ment of  the  pituitary  gland  and  a  modification  of  its 
secretion,  and  a  similar  tendency  to  enlargement  has 
been  observed  clinically  in  cretinism  and  in  other  in- 
stances of  thyroid  deficiency.  If,  then,  insufficiency  of 
thyroid  secretion  is  accompanied  by  some  compensatory 
process  on  the  part  of  the  pituitary  body,  as  appeals 
to  be  the  case,  it  seems  permissible  to  assume  that,  in 
certain  circumstances,  adminivStration  of  thyroid  may 
diminish  the  pituitary  functions  and  lead  to  a  decrease 
in  the  size  of  the  gland. 

However  this  may  be,  there  remains  the  clinical  fact 


ACROMEGALY        •  385 

that  patients  do  frequently  show  some  improvement 
after  taking  thyroid,  especially  with  regard  to  their 
sight,  and  in  the  present  state  of  knowledge  this  method 
is  probably  as  rational  a  mode  of  procedure  as  can  be 
adopted.  Schweinitz  thinks  the  value  of  the  thyroid  is 
enhanced  when  given  in  association  with  mercurial 
inunctions. 

Exposure  to  X-rays  has  been  recommended  as  a 
means  of  causing  pituitary  tumours  to  shrink,  and 
beneficial  results  are  said  to  have  occurred.  Presumably 
the  action  would  be  similar  to  that  which  the  rays 
exert  on  enlarged  thyroid  glands. 

When  the  local  pressure  symptoms  are  great,  the 
question  of  the  possibility  of  obtaining  relief  from  a 
surgical  operation  has  to  be  considered.  The  possi- 
bilities in  this  respect  comprise:  (1)  relief  of  symptoms 
by  a  local  decompressive  operation  to  relieve  the  tension 
in  the  sella  turcica  ;  (2)  drainage  of  a  cyst  should  one 
be  found  to  be  present ;  (3)  partial  removal  of  an 
adenomatous  or  malignant  growth. 

Any  operation  on  the  pituitary  gland  is  necessarily 
one  of  difficulty  and  severity,  and  for  the  various  routes 
and  methods  of  procedure  the  reader  is  referred  to 
Cushing's*  other  special  surgical  works  on  the  subject. 

*  "The  Pituitary  Body  and  its  Disorders." 


CHAPTER   XXXIX 

PROGRESSIVE    LENTICULAR    DEGENERATION 

An  uncommon  disease  under  the  title  of  "  progressive 
lenticular  degeneration,"  which  occurs  in  young  people 
and  tends  to  run  in  families,  has  been  described  by 
Kinnier  Wilson,* 

Symptoms. — Among  the  principal  symptoms  are 
tremors,  muscular  rigidity,  difficulty  and  pain  in  swal- 
lowing, spasmodic  contractions  and  contractures.  The 
patients  are  emotional,  and  mental  symptoms  may  be 
present ;  the  reflexes  in  uncomplicated  cases  are  normal, 
but  they  may  be  modified  if  the  pyramidal  tracts  are 
secondarily  affected. 

Morbid  anatomy. — Bilateral  degeneration  of  the 
lenticular  nucleus,  especially  of  that  part  known  as 
the  putamen,  is  the  principal  lesion  met  with  in  the 
brain,  while,  of  the  other  organs,  cirrhosis  of  the  liver 
which  does  not  cause  any  symptoms  during  life,  and 
is  not  of  syphilitic  or  alcohofic  type,  is  constantly 
present,  and  is  regarded  by  Wilson  as  being  the  probable 
primary  factor  of  the  disease. 

Prognosis  and  treatment. — The  disease  is  pro- 
gressive, but  uncertain  in  its  duration.  An  average 
length  of  time  for  chronic  cases  is  given  as  four  years, 
but  in  some  instances  a  fatal  termination  has  occurred 
in  a  few  months.  The  disease  does  not  react  to  any 
known  form  of  treatment. 

*  Brain,  vol.  xxxiv.,  iiud  Pt.  iv.,  1912. 


386 


CHAPTER   XL 

CEREBRAL     HAEMORRHAGE ;      CEREBRAL 
THROMBOSIS  ;  CEREBRAL  EMBOLISM 

Arterial  supply  of  the  brain. — The  blood  supply 
of  the  brain  is  derived  from  the  internal  carotid  and 
vertebral  arteries.  On  reaching  the  base  of  the  brain 
each  internal  carotid  divides  into  two  brancheti,  which 
are  known  as  the  anterior  and  middle  cerebral  arteries. 
The  anterior  cerebral  arteries  run  forward  and,  passing 
over  the  genu  of  the  corpus  callosum,  continue  their 
course  in  the  median  fissure  to  supply  the  anterior 
part  of  the  frontal  lobes  and  the  inner  surface  of  the 
hemispheres  as  far  back  as  the  parieto-occipital  fissure. 
The  two  anterior  cerebral  arteries  are  united,  soon  after 
their  origin  at  the  base  of  the  brain,  by  a  branch  that  is 
known  as  the  anterior  communicating  artery.  The 
other  branch  of  the  internal  carotid,  namely,  the  middle 
cerebral  artery,  runs  along  the  Sylvian  fissure  until  it 
reaches  the  island  of  Reil,  where  it  divides  into  terminal 
branches  which  supply  the  outer  surface  of  the  hemi- 
sphere. The  middle  cerebral  arteries  are  responsible 
for  the  blood  supply  of  the  whole  of  the  outer  surface 
of  the  cerebral  cortex,  with  the  exception  of  two  narrow 
strips',  one  on  the  anterior  border  of  the  frontal  lobe 
and  another  at  the  lower  border  of  the  temporo-sphenoidal 
and  occipital  lobes.     {See  Fig.  99.) 

As  the  middle  cerebral  artery  runs  across  the  base 
of  the  brain  it  gives  ofi  the  lenticulo-optic  and  lenticulo- 
striate  branches  to  the  corpus  striatum,  which  branches, 
owing  to  the  poorness  of  their  anastomosis,  are  known 

387 


388    DISEASES   OF   THE   NERVOUS   SYSTEM 

as  "  end  arteries."  The  lenticulo-striate  branch  runs 
along  the  outer  side  of  the  lenticular  nucleus  and  sup- 
plies the  region  of  the  internal  capsule.  It  is  notorious 
for  its  liability  to  rupture,  for  which  reason  it  has  been 
called  the  artery  of  cerebral  hcemorrhage. 

Another  result  of  the  poorness  of  anastomosis  of  the 
central  arteries  is  that  any  embolic  or  thrombotic  affec- 
tion of  them  is  likely  to  be  followed  by  permanent  soften- 
ing of  that  part  of  the  brain  which  they  supply. 


Fig.  99.— Area  of  softening  of  the  cortex  due  to  obliteration  of  the 
Sylvian  artery  at  its  commencement.    {Pierre  Mnrie.) 

The  two  vertebral  arteries  run  along  the  surface  of  the 
medulla,  and  on  reaching  the  pons  unite  to  form  the 
basilar  artery;  but  each  vertebral  just  before  uniting 
with  its  fellow  of  the  opposite  side  gives  off  an  important 
branch,  the  posterior  inferior  cerebellar  artery,  which 
runs  obliquely  backwards  round  the  medulla,  to  which 
it  gives  branches,  and  then  passes  on  to  form  an  im- 
portant source  of  blood  supply  of  the  cerebellum.  The 
basilar  artery  supplies  branches  to  the  pons,  and  after 
giving  off  the  anterior  inferior  and  superior  cerebellar 
arteries  it  breaks  up  to  form  the  two  posterior  cerebral 
arteries.  The  posterior  cerebral  arteries  wind  round  the 
cms  cerebri,  and  supply  the  lower  parts  of  the  temporal 


CEREBEAL    HiEMORRHAGE  389 

and  the  occipital  lobes.  Each  artery  also  communicates 
with  the  internal  carotid  by  the  posterior  communi- 
cating artery,  the  circle  of  Willis  being  thus  established 
at  the  base  of  the  brain. 

CEREBRAL  HEMORRHAGE 

Etiology. — ^Arterial  degeneration  and  an  accompany- 
ing rise  of  blood-pressure  are  the  main  factors  concerned 
in  the  production  of  cerebral  hsemorrhage. 

These  two  conditions  are  most  often  associated  with 
chronic  interstitial  nephritis,  and  it  is  during  the  course 
of  this  disease  that  a  large  number  of  cases  of  haemor- 
rhage occur. 

Naturally,  the  causes  which  lead  to  the  final  rupture 
of  a  vessel  are  often  complex  and  difficult  to  analyse, 
since  they  include  all  those  which  tend  to  produce 
arterial  degeneration :  some  of  the  principal  of  these 
are  alcoholic  excess,  chronic  lead-poisoning,  syphilis,  and 
senile  degeneration. 

It  is  particularly  important  to  remember  the  tendency 
shown  by  the  blood-pressure  to  rise  during  the  later 
years  of  middle  life,  even  when  there  are  present  no 
recognizable  signs  of  renal  disease ;  and  no  doubt,  as  Sir 
Clifford  Allbutt  has  strongly  insisted,  a  more  general 
and  timely  recognition  of  this  fact,  combined  with  suit- 
able treatment,  would  lead  to  many  attacks  of  apoplexy 
being  averted  or  postponed. 

The  obliterative  endarteritis  from  syphiUs  more 
often  leads  to  thrombosis  than  to  hsemorrhage,  as  also 
does  the  degeneration  which  accompanies  very  old  age. 

The  arteries  of  men  break  more  often  than  those  of 
women,  and  the  period  in  which  most  cases  occur  is 
from  45  to  65  years  of  age.  Sudden  exertion  may  pre- 
cipitate the  attack,  but  in  a  considerable  number  of 
instances  the  artery  breaks  while  the  patient  is  asleep. 
Haemorrhage  sometimes  takes  place  into  a  tumour  of 
the  brain,  and  it  may  also  be  associated  with  changes 
in  the  blood,  as  in  leucocythaemia,  pernicious  anaemia, 
purpura,   septicaemia,   and   scurvy,   but  the   symptoms 


390    DISEASES   OF   THE   NERVOUS   SYSTEM 

produced  in  these  latter  cases  are  seldom  those  of  typical 
apoplexy. 

Pathology. — The  arteries  in  a  case  of  cerebral 
haemorrhage  generally  present  the  usual  appearances  of 
an  advanced  stage  of  arterio-sclerosis.  Their  walls  are 
rigid  and  brittle,  and  small  aneurysmal  dilatations,  the 
rupture  of  one  of  which  is  the  cause  of  the  haemor- 
rhage, can  frequently  be  seen  with  the  naked  eye  or  a 
magnifying  glass. 

The  appearance  of  the  blood  differs  according  to  the 
length  of  time  which  has  elapsed  since  the  haemorrhage 
occurred.  At  first  there  are  all  the  appearances  of 
freshly  extra vasated  blood,  but,  after  a  clot  has  formed, 
a  gradual  shrinkage  takes  place,  and  the  last  appearances 
may  be  those  of  a  scar  or  a  cyst  with  discoloured  walls 
containing  some  clear  or  brownish  fluid. 

Symptoms. — Symptoms  which  depend  on  de- 
generated vessels  and  high  blood-pressure,  such  as  head- 
ache, giddiness,  tingling  and  numbness  of  a  limb,  are 
often  complained  of  by  people  whose  arteries  are  likely 
to  rupture,  and  sometimes  more  distinct  warnings,  such 
as  a  ptosis,  squint,  or  aphasia  of  passing  duration,  are 
met  with.  Many  such  signs  are  probably  due  to  minute 
haemorrhages,  the  tendency  to  which  may  often  be  seen 
by  looking  at  the  retina,  but  it  is  also  possible  that 
they  may  be  produced  by  arterial  spasm,  though  the 
occurrence  of  spasm  of  cerebral  arteries  is  a  matter 
about  which  there  is  a  good  deal  of  difference  of  opinion. 

A  haemorrhage  causes  symptoms  (1)  by  the  rise 
of  intracranial  pressure  which  it  produces,  and  (2)  by 
destroying  the  function  of  the  nerve  fibres  in  the 
locality  in  which  it  occurs. 

When  a  haemorrhage  is  quite  small  there  may  be 
symptoms  of  local  injury  without  any  disturbance  of 
consciousness,  and  it  is  probably  in  this  way  that  the 
temporary  ptosis  and  squints  sometimes  preceding  a 
more  severe  attack  are  produced. 

On  the  other  hand,  consciousness  may  be  considerably 
disturbed  without  any  permanent  local  effect. 


PLATE  21.— Section  of  brain   showing  the 
anatomy  of  the  internal  capsule. 

A,  Caudate  nucleus  ;     b,  lenticular  nucleus ; 
0,  internal  capsule  ;    d,  optic  thalamus. 


CEREBRAL   HAEMORRHAGE:    SYMPTOMS     391 

Classification  of  symptoms. — It  is  convenient  to  classify 
the  symptoms  into  : 

(1)  General — common  to  all  haemorrhages  of  a 

certain  size,  irrespective  of  their  situation. 

(2)  Local — depending   on    the    position   of    the 

haemorrhage. 

(1)  The  chief  general  symptoms  are  :  loss  of  con- 
sciousness, varying  in  degree  from  confusion  to  coma  ; 
stertorous  breathing,  which  may  be  of  the  Cheyne-Stokes 
type  ;  slow  full  pulse,  subnormal  temperature,  and  loss 
of  power  over  the  sphincters  (this  last  depends  on  the 
depth  of  unconsciousness). 

(2)  The  local  symptoms  necessarily  vary  with  the 
position  of  the  haemorrhage,  which  may  be  anywhere 
in  the  brain,  but  the  cerebral  vessels  which  most  often 
break  are  the  branches  of  the  lenticulo- striate  artery, 
which  come  ofi  directly  from  the  middle  cerebral  and 
pierce  the  base  of  the  brain  to  supply  the  neighbourhood 
of  the  lenticular  nucleus.  These  vessels  are  in  close 
proximity  to  the  motor  fibres  as  they  pass  through  the 
internal  capsule,  and  an  efiusion  of  blood  of  any  size 
in  this  neighbourhood  nearly  always  causes  loss  of 
power  on  the  opposite  side  of  the  body.     (Plate  21.) 

The  haemorrhage  is  usually  large  enough  also  to  cause 
loss  of  consciousness,  and  hence,  in  the  greater  number 
of  cases,  the  symptoms  bear  a  close  resemblance  to  one 
another. 

As  a  typical  example,  it  is  therefore  convenient  to 
discuss  the  results  of  hcemorrhage  and  hemiplegia  resulting 
from  rupture  of  the  lenticulo- striate  artery. 

When  the  attack  takes  place  in  the  daytime  the 
patient  feels  giddy  and,  after  perhaps  reeling  for  a  few 
steps,  sinks  in  a  dazed  manner  to  the  ground  or  on  to 
a  chair,  and  quickly  loses  consciousness.  It  is  usually 
after  this  condition  has  existed  for  some  little  time  that 
he  is  seen  by  the  physician ;  the  patient  is  then  completely 
unconscious,  breathing  stertorously,  and  has  a  full,  slow, 
bounding  pulse  and  a  turgid  countenance.  Perhaps  the 
head  and  eyes  are  persistently  turned  to  one  or  other  side. 


392    DISEASES   OF   THE   NERVOUS   SYSTEM 

Diagnosis. — An  attempt  at  this  stage  must  first 
be  made  to  determine  the  cause  of  the  coma,  but  for  a 
time  it  may  be  impossible  to  arrive  at  any  definite 
conclusion. 

The   chief   conditions   besides   cerebral  haemorrhage 
that   suggest  themselves  in   such   a   case   are  urcemia 
diabetes,  alcohol,   opium  or  other  narcotic,   'postepileptic 
coma,  and  concussion. 

In  urcemia  the  attacks  may  for  a  time  very  closely 
resemble  those  of  cerebral  haemorrhage,  especially  if,  as 
sometimes  happens,  the  power  of  movement  on  the  two 
sides  of  the  body  is  unequal.  Moreover,  cerebral  haemor- 
rhage is  likely  to  occur  in  the  very  people  who  are  also 
liable  to  uraemia.  In  some  cases,  twitchings  and  con- 
vulsions, together  with  the  history  of  the  previous  symp- 
toms, afford  some  assistance,  but  in  others  the  diagnosis 
must  for  a  while  remain  in  doubt. 

Coma  due  to  alcohol  often  gives  rise  to  difficulty, 
and  the  danger  lies  in  pronouncing  a  man  to  be  drunk 
when  he  has  been  the  subject  of  a  haemorrhage  while 
drinking.  The  evidence  of  drink  does  not  exclude  the 
possibility  of  haemorrhage,  and,  when  there  is  the  least 
doubt,  the  only  safe  course  is  to  keep  the  patient  under 
careful  observation  for  a  few  hours. 

The  coma  of  diabetes  generally  comes  on  rather  more 
gradually  than  that  of  haemorrhage.  The  dyspnoea  is 
that  of  the  "  air  hunger  "  type,  and  lacks  the  deep  stertor 
that  accompanies  haemorrhage,  nor  is  there  any  sign  of 
unilateral  paralysis.  If  a  specimen  of  urine  can  be  ob- 
tained the  presence  of  sugar  will  make  the  diagnosis  clear. 

The  signs  of  opium  poisoning  closely  resemble,  in  the 
contracted  pupils  and  deep  coma,  those  of  haemorrhage 
into  the  pons.  Fortunately  there  are  generally  some 
indications  in  the  history  which  lead  to  the  suspicion 
of  poisoning. 

The  coma  which  follows  an  epileptic  fit  is  sometimes 
very  deep,  but  there  is  generally  an  account  of  the 
convulsion  even  if  the  history  of  a  previous  liability 
to  fits  be  not  obtainable. 


CEREBEAL  HEMORRHAGE  :   DIAGNOSIS     393 

In  concussion,  the  difficulty  of  deciding  whether  there 
is  a  meningeal  haemorrhage  may  arise,  for  it  very  rarely 
happens  that  an  intracerebral  artery  ruptures  from 
the  direct  effects  of  a  blow.  When  a  meningeal  artery 
bleeds,  the  signs  are  those  of  an  increasing  pressure  on 
the  brain,  possibly  accompanied  by  convulsions. 

In  the  course  of  the  examination  of  a  case  of  supposed 
cerebral  hasmorrhage,  careful  observation  often  detects 
signs  of  one-sided  weakness.  One  cheek  puffs  out 
loosely,  and  the  arm  and  leg  on  the  same  side  w^hen 
raised  fall  in  a  heavy,  dead  fashion,  which,  in  spite  of 
the  loss  of  consciousness,  obviously  differs  from  that  of 
the  other  side  and  justifies  the  conclusion  that  a  one- 
sided cerebral  lesion  is  the  cause  of  the  trouble. 

The  ground  is  cleared  considerably  when  this  conclu- 
sion can  be  arrived  at,  since  a  one-sided  lesion  coming 
on  suddenly  in  a  man  w^ho  has  up  to  that  time  been  in 
ordinary  health  almost  certainly  indicates  a  vascular 
lesion,  and  so  the  issue  is  narrowed  to  Jicemorrhage, 
thrombosis,  or  embolism. 

Embolism  must  be  excluded  by  the  absence  of  a 
cause,  such  as  valvular  disease  of  the  heart,  and  more- 
over the  deep  loss  of  consciousness  would  at  once  weigh 
heavily  against  it ;  for  although  blocking  of  the  middle 
cerebral  artery  or  one  of  its  branches  will  cause  a  good 
deal  of  mental  confusion,  especially  if  complicated  with 
aphasia,  there  is  nothing  in  its  nature  to  produce  the 
deep  coma  that  accompanies  the  rapid  rise  of  intracranial 
pressure  consequent  on  the  effusion  of  a  quantity  of 
blood. 

In  the  diagnosis  between  thrombosis  and  hemorrhage, 
often  a  most  difficult  matter,  the  main  points  to  be 
considered  are — 

1.  Loss  of  consciousness. — The  existence  of  extensive 
paralysis  without  any  definite  loss  of  the  senses  is  highly 
suggestive  of  thrombosis,  for  a  sudden  effusion  which 
is  large  enough  to  cause  hemiplegia  is  most  likely  also 
to  raise  the  intracranial  pressure  sufficiently  to  cause 
loss  of  consciousness.     Of  course,  an  exception  to  this 


394    DISEASES   OF   THE   NEKVOUS   SYSTEM 

general  rule  may  occur  when  a  small  hsemorrliage  takes 
place  somewhere  exactly  in  the  course  of  the  motor 
fibres; 

2.  Onset  of  paralysis. — In  the  case  of  haemorrhage 
the  onset  of  paralysis  is  generally  sudden  and  does  not 
show  signs  of  extension,  for  the  damage  is  usually  all 
done  in  a  very  few  minutes.  On  the  other  hand,  a 
paralysis  which  progresses  in  depth  and  distribution 
over  a  period  of  some  hours,  especially  if  there  is  no 
loss  of  consciousness,  is  very  characteristic  of  thrombosis 
in  which  the  clot  is  extending  from  the  original  thrombus 
into  neighbouring  vessels.  Before  the  onset  of  paralysis 
there  may  have  been  numbness,  tingling,  or  a  passing 
weakness  in  a  limb,  signifying  that  the  blood  supply  was 
very  deficient  before  the  occlusion  finally  occurred. 

3.  The  vascular  system. — The  association  of  an  hyper- 
trophied  heart  with  degenerated  arteries  is  in  favour 
of  haemorrhage.  High  arterial  tension  also  disposes  to 
haemorrhage,  and  further  evidence  may  be  obtained  from 
signs  of  the  existence  of  chronic  interstitial  nephritis. 

4.  Age. — In  the  very  old,  thrombosis  is  more  likely 
to  occur  than  haemorrhage.  In  the  young  and  in  early 
middle  life,  say  under  40,  cerebral  haemorrhage  is  rare, 
while  thrombosis  is  comparatively  frequent  from  syphi- 
litic endarteritis. 

Treafment  during  the  eai*ly  stage. — As  soon 
as  the  diagnosis  of  cerebral  haemorrhage  has  been  made 
it  is  necessary  to  proceed  at  once  to  treat  the  patient. 

In  rare  instances,  where  the  patient  is  seen  at  the 
moment  the  bleeding  is  taking  place,  prompt  venesection 
and  pressure  on  the  carotid  arteries  might  be  of  use  ; 
but,  as  a  general  rule,  the  time  for  these  measures  has 
gone  before  any  opportunity  of  treating  the  patient 
arises.  The  best  method  then  of  trying  to  prevent  further 
oozing  is  to  keep  the  patient  at  absolute  rest,  and  to 
calm  down  the  circulation  by  relaxation  of  the  bowels 
to  a  moderate  degree,  such  as  can  be  obtained  by  calomel 
(gr.  ii  to  v)  or  croton  oil  (n\i  to  i),  followed  if  necessary 
by  an  enema. 


CEREBRAL   HAEMORRHAGE:   PROGNOSIS    395 

The  patient's  head,  upon  which  an  ice-bag  may  be 
placed,  should  be  raised  and  turned  rather  to  one  side, 
in  which  position  the  stertor  is  often  diminished.  Eor 
the  first  few  hours  no  food  is  necessary ;  after  that  a 
little  milk  may  be  given.  When  necessary  the  urine 
should  be  drawn  off,  great  care  being  taken  to  keep  the 
skin  clean  and  dry. 

Prognosis. — The  next  step  which  it  is  usually 
necessary  to  take  is  to  give  an  opinion  as  to  the  danger 
to  life,  and  in  this  connection  the  chief  points  to  observe 

(1)  The  de'pih  of  the  co7na. 
_  (2)  The  course  of  the  temperature. 

(3)  Whether   conjugate   deviation   of    the   head 
and  eyes  is  present. 

A  deep  loss  of  consciousness  suggests  that  either  the 
haemorrhage  is  large  or  situated  in  some  specially 
important  part  of  the  brain,  as,  for  instance,  the  neigh  - 
bourhood  of  the  pons  or  medulla  ;  in  both  cases  life  is 
in  danger.  Of  particularly  evil  significance  is  a  coma 
which  deepens  progressively,  for  it  usually  indicates  that 
the  bleeding  is  still  going  on,  or  that  the  blood  is  finding 
its  way  into  the  lateral  ventricles.  Generally  speaking, 
if  there  are  no  distinct  signs  of  returning  consciousness 
in  twenty- four  hours  the  outlook  is  bad.  On  the  other 
hand,  slight  loss  of  consciousness  and  signs  that  the 
coma  is  beginning  to  pass  off  are  both  satisfactory 
indications. 

Immediately  after  the  haemorrhage  has  taken  place 
there  is  a  sudden  fall  in  the  temperature  to  two  or  three 
degrees  below  normal,  which  is  followed  by  a  rise  above 
normal  when  the  preliminary  shock  has  passed  off.  If 
this  rise  keeps  within  the  limits  of  one  to  two  degrees 
above  normal  the  outlook  is  favourable ;  but  if  the 
temperature  is  raised  above  this  for  any  length  of  time, 
and  shows  signs  of  being  raised  still  more,  it  is  a  sign 
that  the  patient  will  probably  die. 

Conjugate  deviation  of  the  eyes  and  head,  especially 
if  it  persists,  is  a  sign  of  bad  omen. 


396    DISEASES   OF   THE  NERVOUS   SYSTEM 

Otlier  symptoms,  such  as  the  state  of  the  pupils 
and  the  presence  of  Cheyne-Stokes  breathing,  may  also 
help  in  making  a  prognosis. 

Signs  of  hemiplegia. — With  return  of  con- 
sciousness the  local  effects  of  the  lesion  become  more 
apparent,  and  when  the  haemorrhage  is  in  the  region  of 
the  internal  capsule  there  are  often  sensory  as  well  as 
motor  symptoms. 

1.  Motor  symptoms. — There  will  most  likely  be  loss 
of  power  of  the  upper-neurone  type  for  voluntary  move- 
ments in  the  face,  arm,  and  leg  on  the  opposite  side  to 
the  lesion. 

Face. — In  examining  the  face  there  are  three  sets 
of  movements  to  be  considered,  viz.  : 

(1)  Unilateral    voluntary    movements,  i.e.   volun- 

tary movements  that  are  habitually  made 
on  one  side  of  the  face,  e.g.  drawing  up 
the  corner  of  the  mouth. 

(2)  Bilateral  voluntary  movements,  i.e.  voluntary 

movements  in  which  both  sides  of  the  face 
are  habitually  put  into  action  at  the  same 
time,  e.g.  closing  the  eyes  and  wrinkling 
the  brow, 

(3)  Emotional  movements,  e.g.  those  of  laughing 

and  crying,  into  which  volition  only  enters 
to  a  slight  degree. 

In  hemiplegia  these  three  sets  of  movements  are 
affected  in  very  different  degrees. 

The  unilateral  voluntary  movements  are  the  most 
highly  specialized,  and  the  path  by  which  impulses  for 
their  production  can  pass  from  the  motor  cortex  to  the 
muscles  is  confined  to  the  direct  course  of  the  motor 
fibres.  Interruption  of  these  fibres  leaves  no  alternative 
route,  hence  these  particular  movements  are  lost. 

Bilateral  voluntary  movements  are  less  specialized, 
and  there  is  still  some  communication  possible  between 
the  nuclei  of  their  nerves  on  the  two  sides.  When, 
therefore,  the  path  from  the  cortex  is  interrupted  on  one 
side,  it  is  still  possible  to  obtain  impulses  through  the 


HEMIPLEGIA  397 

communication  with  the  nucleus  on  the  other  side,  and 
so  in  hemiplegia  the  voluntary  bilateral  movements, 
though  often  temporarily  weakened,  do  not  suffer  to 
such  an  extent  as  the  unilateral  ones.  The  patient 
is  thus  enabled  to  wrinkle  the  brow,  frown,  and  close 
the  eyes,  even  though  he  cannot  move  the  corner  of 
his  mouth. 

Reference  has  already  been  made  to  the  lateral 
deviation  of  the  head  and  eyes  which  may  take  place 
in  severe  cases  (p.  395). 

Emotional  movements  are  still  less  diminished,  and 
the  corner  of  the  mouth  often  moves  freely  during 
laughing  or  crying  when  it  cannot  be  moved  by  the  will. 

The  tongue,  when  put  out,  usually  deviates  towards 
the  paralysed  side,  and  on  phonation  the  two  halves  of 
the  soft  palate  are  unequally  drawn  up. 

The  arm  is  generally  deeply  paralysed,  the  finer 
movements  of  the  fingers  suffering  more  than  the  coarser 
ones  of  the  elbow  and  shoulder. 

The  leg  is  usually  less  deeply  paralysed  than  the  arm, 
and  is  nearly  always  the  first  to  show  signs  of  returning 
power. 

In  hemiplegia  of  organic  origin,  Babinski  has  shown 
that  if  the  patient  lies  on  his  back  with  his  legs  widely 
separated  and  attempts  to  assume  a  sitting  position, 
the  paralysed  leg  is,  in  the  attempt,  lifted  higher  from 
the  floor  than  the  sound  limb — a  phenomenon  which 
forms  one  of  the  distinguishing  features  between  organic 
and  functional  disease. 

The  movements  of  the  trunk  are  for  the  most  part 
bilateral,  and,  although  slight  differences  can  be  detected 
between  the  respiratory  movements  of  the  two  sides, 
they  are  not  usually  of  special  clinical  importance. 

2.  The  reflexes. — The  tendon  reflexes  on  the  paralysed 
side  are  exaggerated,  as  is  the  rule  in  upper  neurone 
lesions,  and  after  a  time  there  is  sometimes  an  increase 
in  those  of  the  opposite  side  also.  In  the  leg  the  knee- 
jerk  and  tendo-Achillis  jerk  are  very  active,  and  ankle- 
clonus   can   usually   be   obtained.     The   plantar   reflex 


398    DISEASES   OF   THE   NERVOUS   SYSTEM 

generally  gives  a  typical  extensor  response.  The  super- 
ficial abdominal  reflexes  on  the  paralysed  side  may  be 
lost. 

After  return  of  consciousness,  unless  there  is  con- 
siderable impairment  of  the  mental  faculties,  the  organic 
reflexes,  such  as  those  of  the  bladder  and  deglutition, 
are  usually  under  control. 

3.  Sensory  changes. —  Hemianesthesia  may  be  present 
when  the  sensory  part  of  the  internal  capsule  is  injured, 
and,  as  the  fibres  for  vision  from  the  occipital  lobes 
also  pass  over  the  same  part  of  the  internal  capsule, 
hemianofia  is  often  an  accompanying  symptom.  In 
the  majority  of  cases  sensation  is  eventually  restored, 
and  the  hemianopia  also  passes  off. 

4.  Mental  changes. — There  is  generally  some  degree 
of  enfeeblement  of  the  mind  after  a  "  stroke,"  and  it 
is  usually  safe  to  predict  that  the  patient  will  not  retain 
the  same  ability  for  business  that  he  possessed  before 
the  occurrence  of  the  haemorrhage. 

Course  of  events. — ^As  lime  goes  on,  the  signs 
which  accompany  degeneration  of  upper  neurone  fibres 
become  more  apparent.  The  tendon  reflexes  become 
further  increased,  and  ankle-clonus  and  the  extensor 
response  of  the  plantar  reflex  become  apparent  if  they 
were  not  so  before. 

There  is  a  tendency  for  the  limbs  to  become  rigid 
and  to  develop  contractures.  At  the  same  time  there 
is  no  obvious  wasting,  except  such  as  may  be  accounted 
for  by  disuse,  nor  can  any  changes  be  observed  in  the 
electrical  reactions  of  the  muscles. 

The  onset  of  rigidity  and  contractures  causes  hemi- 
plegic  patients  to  assume  a  characteristic  attitude  and 
gait. 

In  the  arm  the  contractures  of  flexors  and  adductors 
are  stronger  than  those  of  extensors  and  abductors,  so 
that  the  arm  is  drawn  in  close  to  the  side,  pronatcd,  and 
flexed  at  the  elbow,  wrist,  and  fingers.  In  the  leg 
extensor  spasm  generally  predominates,  so  that  the 
rigidity  prevents  the  joints  from  being  freely  used,  and  in 


PLATE  22. -Characteristic  gait  in  hemiplegia. 

The  rigid  and  weakened  left  leg  is  stiffly  circumducted  from  the  hip. 

Note  also  the  characteristic  position  of  the  paralysed  arm. 


HEMIPLEGIA  399 

walking  the  whole  limb  is  stiffly  swung  round  in 
the  arc  of  a  circle  from  the  hip,  i.e.  circumducted 
(Plate  22). 

The  hypotheses  which  have  been  brought  forward 
to  account  for  this  rigidity  and  the  methods  necessary 
to  adopt  in  order  to  avert  it  are  discussed  at  p.  39. 

Involuntary  movements. — Hemiplegic  conditions 
are  sometimes  accompanied  by  involuntary  movements 
known  as  post-hemiplegic  chorea  and  athetosis.  Such 
movements  appear  more  prone  to  occur  in  lesions 
of  the  thalamus  and  its  neighbourhood,  and  are  appar- 
ently due  to  release  from  control  with  consequent  over- 
activity of  subsidiary  motor  systems  {see  p.  10).  These 
movements  are  very  apt  to  arise  in  the  hemiplegias 
and  diplegias  of  childhood,  but  their  onset  may  be 
postponed  for  a  considerable  time  after  the  date  of  the 
original  lesion. 

Post-hemiplegic  chorea  is  characterized  by  tremors 
or  irregular  movements  in  the  arm  or  leg  of  the  para- 
lysed side. 

Athetosis  is  characterized  by  involuntary  move- 
ments which  are  generally  most  pronounced  in  the  hand 
or  foot,  but  occasionally  the  whole  limb  may  take  part, 
as  also  may  the  face. 

In  the  hand,  the  fingers  move  in  a  slow  manner, 
and  are  alternately  flexed  and  hyperextended.  All  the 
fingers  do  not  move  equally,  but  one  or  more  at  a  time 
are  hyperextended  in  a  fashion  which  it  is  not  possible 
to  imitate.  In  some  instances  the  movements  go  on 
almost  continually,  but  in  others  they  are  often  started 
by  an  attempt  to  move  the  Hmb  voluntarily.  In 
severe  cases  the  arm  may  be  thrown  about  in  such  a 
fashion  that  it  becomes  necessary  to  bind  it  to  the 
patient's  side. 

Associated  movements  are  also  met  with  in 
hemiplegics.  The  paralysed  arm  is  often  strongly  drawn 
across  the  chest  when  the  patient  yawns  or  breathes 
deeply,  and  the  movement  of  one  leg  may  be  accom- 
panied by  some  movement  of  the  other. 


400    DISEASES   OF   THE  NERVOUS   SYSTEM 

When  all  signs  of  improvement  in  the  power  of  the 
limbs  cease,  and  rigidity  makes  its  appearance,  there  is 
not  much  hope  of  any  further  improvement  so  far  as 
recovery  of  the  functions  of  the  injured  neurones  is 
concerned.  Treatment  must,  however,  on  no  account 
be  abandoned,  for  by  improving  the  function  of  fibres 
that  remain,  and  by  warding  off  rigidity  and  contrac- 
tures, much  can  be  done  to  render  the  limb  a  useful 
one.  If  the  methods  described  at  p.  41  are  per- 
severed with,  marked  benefit  can  often  be  obtained ; 
and  it  must  always  be  remembered  that  the  difference 
between  a  helpless  limb  and  one  that  can  be  used, 
however  slightly,  is  of  the  greatest  importance  to  the 
patient. 

Pain. — In  some  cases  pain  in  the  paralysed  limbs  is 
a  very  distressing  symptom.  It  is  very  apt  to  occur  in 
association  with  lesions  of  the  thalamus.  As  explained 
on  p.  61,  it  may  be  due  to  over-activity  of  the  thalamus 
rather  than  to  persistent  irritation  of  sensory  fibres, 
which  is  an  explanation  frequently  suggested. 

Haemorrhage  into  the  pons  and  crus  cerebri, 
— Haemorrhage  into  the  pons  is  usually  fatal  within  a 
few  hours. 

Deep  coma,  contracted  pupils,  and  hyperpyrexia 
are  among  the  characteristic  signs.  Convulsions  may 
also  occur.  There  is  often  paralysis  on  both  sides  of  the 
body,  since  a  haemorrhage  of  any  size  will  injure  both 
sets  of  motor  fibres. 

If,  however,  the  haemorrhage  is  small  and  situated 
at  the  upper  part  of  the  pons  or  in  the  crus  cerebri,  it 
is  possible  to  get  "  crossed  paralysis,"  i.e.  of  the  face  on 
one  side  and  of  the  arm  and  leg  on  the  other,  owing  to 
the  fibres  of  this  nerve  crossing  at  a  higher  level  than 
those  for  the  arm  and  leg,  which  do  not  decussate  till 
they  reach  the  medulla. 

The  fibres  to  the  ocular  muscles  may  also  be  affected 
in  lesions  of  the  pons. 

The  association  of  coma  and  contracted  pupils  makes 
it  necessary  to  bear  in  mind  the  possibility  of  opium- 


CEREBRAL    THROMBOSIS 


401 


poisoning  when  tlie   paralysis   is   not  obvious  and   no 
history  of  the  case  is  available. 

Sumniary   of   the  causes  of  hemiplegia. — The 

symptoms  of  hemiplegia  following  cerebral  haemorrhage 

as  described  in  the  previous  paragraphs  will  serve  as  a 

general  example,  but  it  is  also  necessary  to  remember 

the  other  principal  causes  of  this  condition,  since  the 

patient  may  be  seen  for  the  first  time  long  after  the 

occurrence  of  the  original  lesion. 

The  causes  of  hemiplegia  may  be  classified  somewhat 

as  follows  : 

f  XT  1  (  Meningeal. 

I  Haemorrhage,  w  ^  v    i 

J  I  intracerebral. 

I  Thrombosis. 
\^  Embolism. 

f  f  Localized,  or  especially 

J  Meningitis.     -|      intense      over      the 
I  (^     motor  areas. 

\  Acute  encephalitis. 
.     Pressing  on  the  motor  tracts. 
.     Degenerative  processes   of  the   brain, 

e.g.  sclerosis. 
.     In  uraemia,   the  effects  of  the  poison 
are  sometimes  especially  visited  on  the 
motor  areas,  and  a  hemiplegia  which 
is  often  diflBcult  to  distinguish  from 
that   of   cerebral  haemorrhage  may 
occur. 
Temporary  hemiplegia,  presumably  due 
to  exhaustion,  may  follow  a  severe 
epileptic  fit. 
Hemiplegia  is  a  not  uncommon  mani- 
festation of  hysteria. 


1.    Vascular  lesions 


2.  Inflammations 

3.  Tumotirs     ... 

4.  Degenerations 

5.  Toxcemias   ... 


6.  Exhaustion     follow- 

ing convulsions 

7.  Functional 

hemiplegia 


CEREBRAL  THROMBOSIS 
Etiology. — The  chief  conditions  which  lead  to 
thrombosis  are  atheroma  of  the  vessels  and  a  feehle  circu- 
lation ;  hence,  thrombosis  is  more  likely  to  occur  in 
advanced  years,  when  the  vessel  walls  are  thickened  and 
the  circulation  slow.  On  the  other  hand,  in  the  later 
part  of  middle  life,  when  the  arteries  are  apt  to  be 
weakened,  while  the  force  of  the  heart  still  remains 
strong,  there  is  a  greater  liability  to  haemorrhage. 
2a 


402    DISEASES   OF   THE    NERVOUS   SYSTEM 

Among  the  principal  causes  of  arterial  degeneration 
which  may  eventually  lead  to  thrombosis  are  syphilis, 
alcohol,  and  Bright's  disease,  and  of  these  syphilis  is  the 
most  important.  The  atheroma  of  Bright's  disease  is 
more  likely  to  be  associated  with  haemorrhage  than  with 
thrombosis. 

Syphilitic  endarteritis  is  the  commonest  cause  of  the 
cases  of  cerebral  thrombosis  that  are  met  with  before 
the  occurrence  of  the  degenerative  changes  of  senility. 
The  proliferation  of  the  tissues  of  the  arterial  coats 
gradually  narrows  the  lumen  of  the  vessel  until  finally 
the  flow  of  blood  is  checked  and  the  parts  beyond  are 
deprived  of  their  nutrition. 

In  some  instances  the  cause  of  the  thrombosis  depends 
upon  changes  in  the  composition  of  the  blood.  This  is 
the  case  in  thrombosis  associated  with  severe  anaemias 
and  with  some  varieties  of  septicaemia.  Allusion  has 
already  been  made  to  infective  processes  as  a  cause 
of  thrombosis  in  infantile  paralysis  and  the  cerebral 
palsies  of  children. 

Symptoms. — The  symptoms  vary  according  to  the 
mode  of  onset  and  the  locality  of  the  vessel  that  is 
blocked. 

1.  Gradual  onset. — When  the  lumen  of  the  vessels 
becomes  narrowed  gradually,  the  first  symptoms  are 
likely  to  be  those  due  to  diminished  blood  supply  to 
certain  parts  of  the  brain,  i.e.  to  cerebral  anaemia ;  the 
function  of  the  part  is  then  apt  to  be  impaired  without 
being  altogether  lost,  and  may  perhaps  again  be  restored 
as  some  variation  takes  place  in  the  state  of  the  local 
circulation.  Thus,  before  there  are  signs  of  complete 
thrombosis,  there  may  be  w^arnings  in  the  guise  of  tem- 
porary failure  of  power  in  a  limb,  an  ocular  muscle,  or 
an  eyelid,  or  there  may  perhaps  be  some  loss  of  memory, 
with  a  temporary  condition  of  partial  aphasia  ;  while 
in  other  cases  the  patients  complain  of  tingling,  numb- 
ness, and  giddiness.  One  day  matters  may  go  farther  : 
a  clot  forms,  and  the  area  supplied  by  the  vessel  is  cut 
off  from  the   circulation.     With   a   gradual   onset  the 


CEREBRAL    THROMBOSIS  403 

general  symptoms  of  cerebral  disturbance  are  not  likely 
to  be  great,  and  in  many  instances  consciousness  is 
never  lost. 

2.  Sudden  onset. — A  more  sudden  onset  is  the  char- 
acteristic of  other  cases  where  the  function  of  the  part 
has  not  been  perceptibly  impaired  before  the  vessel 
becomes  completely  blocked.  Paralysis  of  one  or  more 
limbs  sets  in  suddenly,  and  the  general  cerebral  disturb- 
ance is  then  often  sufficiently  great  to  cause  loss  of 
consciousness,  though  the  coma  is  seldom  so  deep  as 
that  of  a  haemorrhage  causing  symptoms  of  a  correspond- 
ing extent.  Some  idea  of  the  effects  of  thrombosis  of 
different  arteries  and  their  branches  can  be  gauged 
from  the  diagram  which  shows  the  area  of  softening  after 
blocking  of  the  middle  cerebral  (Fig.  99,  p.  388). 

If,  as  frequently  happens,  a  branch  of  the  middle 
cerebral  artery  to  the  motor  cortex  is  blocked,  there  will 
be  loss  of  power  corresponding  to  the  extent  of  brain 
affected.  If,  for  example,  the  branch  that  is  blocked 
happens  to  be  that  supplying  the  motor  area  of  the 
cortex  in  which  movements  of  the  arm  are  represented, 
the  limb  will  be  paralysed,  and  will  also  very  likely  be 
subject  to  attacks  of  localized  convulsions  owing  to  the 
irritation  of  the  cerebral  cortex. 

When  a  clot  has  formed  it  frequently  extends  into 
other  branches,  and  thus  a  monoplegia  may  in  a  few 
hours  be  succeeded  by  a  hemiplegia. 

If,  however,  the  main  trunk  of  the  middle  cerebral 
artery  is  blocked,  a  complete  hemiplegia  results  from  the 
first,  and  to  this,  if  the  lesion  is  on  the  left  side  of  the 
brain,  is  added  aphasia. 

An  equally  severe  hemiplegia,  with  perhaps  hemian- 
sesthesia  and  hemianopia,  may  also  result  from  throm- 
bosis of  the  vessels  supplying  the  motor  fibres  of  the 
internal  capsule,  but  speech  is  not  in  these  circumstances 
often  permanently  lost. 

Where  the  tendency  to  thrombosis  is  widespread, 
loss  of  memory  and  general  mental  failure  may  be  the 
most  prominent  features  of  the  case,- 


404    DISEASES   OF   THE  NERVOUS   SYSTEM 

Thrombosis  of  the  posterior  inferior  cerebellar  artery 
also  causes  characteristic  symptonis,  which  usually  in- 
clude some  degree  of  incoordination  of  the  limbs  on  the 
same  side  as  the  lesion,  nystagmus,  strabismus,  and 
disturbances  of  sensation  over  the  half  of  the  face 
on  the  side  of  the  lesion,  with  diminished  sensation  to 
pain  and  temperature,  and  impaired  position-sense  on 
the  hmbs  and  trunk  on  the  side  opposite  the  lesion. 

Pathology. — An  area  of  the  brain  that  is  deprived 
of  its  blood  supply  becomes  softened  and  necrosed. 
Sometimes  it  remains  pale,  but  often  there  is  some 
transudation  of  blood,  as  in  the  case  of  infarcts  of  other 
organs  ;  and  pale,  red,  and  yellow  softening  are  spoken 
of,  according  to  the  coloration  produced  by  the  blood. 
In  late  stages  a  cyst  or  a  scar  may  represent  the  area 
of  disease. 

Prognosis. — In  giving  a  prognosis  of  the  results 
of  thrombosis,  it  is  necessary  to  try  to  form  a  mental 
picture  of  what  is  taking  place  at  the  seat  of  the  disease. 
The  blockage  of  an  artery  causes  total  ancemia  of  any 
portion  of  brain  which  happens  to  be  entirely  dependent 
on  it  for  its  nutrition,  and  'partial  ancemia  of  the  parts 
around,  which  can  still  obtain  some  nutrition  from 
collateral  sources. 

Hence  the  first  effect  is  destruction  of  the  function 
of  the  nerve  cells  in  the  area  which  the  vessel  wholly 
supplies,  and  a  lowering  of  the  function  of  those  parts 
on  the  border  of  that  area  which  obtain  some  of  their 
blood  supply  from  elsewhere.  Unless  the  circulation  to 
the  part  which  is  wholly  dependent  on  the  thrombosed 
artery  is  restored  quickly,  the  nervous  tissue  will  disinte- 
grate and  be  incapable  of  repair.  On  the  other  hand, 
the  function  of  the  parts  in  the  immediate  vicinity, 
depressed  as  the  first  effect  of  the  arterial  blockage, 
improves  to  a  considerable  extent  as  the  collateral  cir- 
culation becomes  better  established.  So  that  when, 
after  a  few  days,  there  is  no  return  of  power,  it  may 
be  fairly  assumed  that  permanent  damage  has  been 
done,   though  a   certain  degree  of   recovery  may  still 


CEREBRAL    THROMBOSIS  405 

take  place  as  the  function  of  the  surrounding  part 
improves. 

Thrombosis  of  the  vessels  supplying  the  region  of 
the  internal  capsule  is  especially  apt  to  be  followed 
by  permanent  softening,  for  the  collateral  circulation 
of  that  part  of  the  brain  is  very  poor  and  can  only  be 
very  feebly  maintained  from  the  terminal  branches  of 
the  cortical  arteries. 

The  outlook  is  bad  in  those  cases  in  which  gradual 
failure  of  the  mind  signifies  a  widespread  softening. 

Diagnosis. — The  diagnosis  from  cerebral  haemor- 
rhage has  been  considered  earlier  in  the  present  chapter 
(p.  393). 

Treatment. — If  the  danger  is  recognized  in  time, 
as  it  may  be  when  the  circulation  is  weak  and  the  arteries 
are  obviously  thickened,  much  can  be  done  to  avert 
thrombosis  by  increasing  the  rate  of  the  blood-flow  and 
by  modifying  the  nutrition  of  the  vessel  walls ;  but, 
once  the  clot  has  formed,  no  method  of  treatment  will 
ensure  its  dispersal,  nor  can  any  drug  restore  the  vitality 
of  a  patch  of  softened  brain. 

In  those  who  have  sjrphilitic  endarteritis,  a  course 
of  mercury  and  iodides  would  doubtless  often  prevent 
catastrophe;  but  too  often  they  seek  no  treatment  till 
the  event  has  taken  place,  and  the  most  that  can  usually 
then  be  done  is  to  try  to  prevent  a  recurrence. 

In  every  case  of  recent  thrombosis  due  to  syphilitic 
disease  of  the  arteries  it  is  a  good  plan  to  prescribe 
antisyphilitic  treatment,  and  afterwards,  with  a  view 
of  anticipating  recurrence,  to  give  regular  courses  of 
iodide  at  stated  intervals,  to  which,  if  it  seems  desir- 
able at  any  time,  injections  of  salvarsan  or  a  course 
of  mercury  may  be  added. 

During  the  attack  the  patient  should  be  kept  quiet 
and  fed  lightly.  Care  should  be  taken  not  to  purge 
severely  and  not  to  adopt  any  other  measures  that  are 
likely  to  depress  the  force  of  the  circulation,  which  may 
often  be  improved  with  advantage  by  a  little  digitalis 
or  other  suitable  cardiac  stimulant. 


406    DISEASES   OF   THE   NERVOUS   SYSTEM 

CEREBRAL   EMBOLISM 

Etiology. — Embolism  is  nearly  always  associated 
with  endocarditis  of  the  aortic  or  mitral  valve.  Occa- 
sionally it  arises  from  detached  portions  of  clot  from 
an  aneurysmal  sac. 

In  chronic  endocarditis,  mitral  stenosis  is  the  form 
most  likely  to  cause  embolism,  but  in  the  acute  infective 
forms  a  fragment  may  be  dislodged  from  either  the  aortic 
or  mitral  cusps. 

Emboli  are  on  the  whole  more  likely  to  pass  up  the 
left  carotid  artery,  and  so  it  is  more  common  to  get 
a  lesion  of  the  left  than  the  right  side  of  the  brain. 

Symptoms. — The  onset  is  quite  sudden  and  is 
accompanied  by  symptoms  which  vary  according  to  the 
position  in  which  the  clot  lodges.  If,  as  often  happens, 
the  main  branch  of  the  middle  cerebral  artery  on  the 
left  side  is  blocked,  hemiplegia  and  aphasia  immediately 
result.  In  such  a  case  the  senses  are  often  lost,  but  there 
are  seldom  the  depth  of  coma,  stertorous  breathing,  and 
disturbance  of  temperature  that  are  met  with  in  cerebral 
haemorrhage. 

Softening  of  the  cortex  wall  probably  be  accompanied 
by  convulsions,  but  these  will  be  absent  when  the  ob- 
struction occurs  in  the  central  branches  of  the  artery 
after  they  have  left  the  main  stem,  though  the  hemi- 
plegia may  be  complete  from  softening  of  the  fibres  of 
the  internal  capsule. 

Wlien  only  a  small  branch  of  the  middle  cerebral 
is  blocked  the  symptoms  are,  of  course,  less  severe. 
Embolism  of  the  posterior  cerebral  artery  causes  hemi- 
anopia,  and  (according  to  Marie)  visual  aphasia  also. 

If  thrombosis  starts  from  the  original  clot  and  extends 
along  the  vessels,  the  sudden  symptoms  at  the  beginning 
are  followed  by  others  of  more  deliberate  progress. 

Prognosis. — What  has  been  said  regarding  the 
prognosis  in  cases  of  cerebral  thrombosis  may  be  in  a 
large  measure  applied  to  cerebral  emboHsm.  In  both, 
the  restoration  of  cerebral  function  depends  upon  the 


CEREBKAL    EMBOLISM  407 

possibility  of  the  rapid  re-establishment  of  the  circula- 
tion, for  when  softening  has  once  occurred  in  a  part,  its 
function  cannot  be  regained,  though  it  may  perhaps 
be  partially  compensated  by  the  action  of  other  parts. 

Diagnosis. — The  diagnosis  between  haemorrhage, 
thrombosis,  and  embolism  is  discussed  at  p.  393. 

Treatment. — The  clot  cannot  be  modified  in  any 
way.  The  after-treatment  is  that  which  is  detailed  lor 
hemiplegia,  and  consists  in  massage  and  correction  of 
faulty  positions  of  the  paralysed  limbs,  while  the  patients 
must  be  encouraged  to  develop  any  return  of  voluntary 
power  that  may  occur; 


CHAPTER  XLI 

THROMBOSIS    OF   CEREBRAL    SINUSES 

Thrombosis  of  the  cerebral  sinuses  may  arise  as  the 
result  of  general  diseases,  e.g.  severe  anaemia,  wasting 
and  diarrhoea  (especially  in  infants),  and  typhoid  fever, 
or  it  may  take  place  after  a  local  injury.  In  these  cases 
the  thrombosis  is  often  called  "  simple,"  as  opposed  to 
the  infective  variety,  which  is  the  more  important, 
and  which  arises  as  the  result  of  some  near  or  distant 
septic  condition.  Suppuration  of  the  middle  ear  or  of 
the  nasal  sinuses  is  the  most  important  local  cause  of 
infection. 

Thrombosis  of  the  lateral  sinus. — This  is  espe- 
cially apt  to  be  associated  with  chronic  suppuration  of  the 
middle  ear,  and  it  may  coexist  with  cerebral  abscess. 

The  chief  local  signs  are  pain,  oedema,  and  distension 
of  veins  over  the  mastoid  region,  with  a  sense  of  hardness 
over  the  course  of  the  internal  jugular  vein,  down  which 
the  clot  frequently  extends.  The  general  symptoms  are 
mainly  those  of  septic  infection,  viz.  high  temperature, 
rapid  pulse,  and  delirium. 

This  condition  gives  cause  for  grave  anxiety.  The 
best  mode  of  treatment  is  to  open  up  the  sinus  and  clear 
out  the  infective  clot,  and  at  the  same  time  to  deal 
with  an  abscess  should  it  be  present. 

Thrombosis  of  the  cavernous  sinus.  —  Sir 
StClair  Thomson  has  shown  *  that  thrombosis  of  the 
cavernous  sinuses  is  especially  liable  to  follow  chronic 
suppuration  of  the  nasal  cavities. 

*  Trans,  of  Med.  Soc.  of  London,  1906. 
408 


THEOMBOSIS    OF    CAVERNOUS    SINUS    409 

The  main  general  symptoms  which  indicate  the  onset 
are,  according  to  Sir  StClair  Thomson,  acute  headache, 
delirium,  and  drowsiness,  with  sickness,  high  tempera- 
ture, and  raj^id  23ulse.     The  headache  is  generally  severe, 


Fig.  100.— CEdema  and  exophthalmos  in  a  case  of 
thrombosis  of  the  cavernous  sinus.  (Sir  StClair 
Thomson.) 


and  the  pain  may  be  referred  to  the  side  of  the  head, 
to  the  back  of  the  ear,  or  to  the  frontal  region. 
With  these  there  are  generally  some  ocular  symptoms, 
consisting  chiefly  of  papillary  oedema,   chemosis,   and 


410    DISEASES   OF   THE   NERVOUS   SYSTEM 

exophthalmos,  to  which  may  be  added  ulceration  of  the 
cornea  and  paralysis  of  ocular  muscles  from  implication 
of  the  first  division  of  the  trigeminal  nerve  and  of  the 
motor  nerves  in  their  passage  along  the  outer  wall  of 
the  sinus.     (Fig.  100.) 

Swelling  of  the  optic  disc  is  also  often  found,  but, 
if  the  case  runs  its  course  rapidly,  many  of  the  ocular 
symptoms  do  not  develop. 

The  prognosis  in  a  case  of  thrombosis  of  the  cavernous 
sinus  is  very  grave,  and  still  more  so  if  the  condition  of 
the  eyes  indicates  bilateral  disease.  Suggestions  and 
attempts  have  been  made  to  reach  these  sinuses,  but 
they  have  hitherto  met  with  little  success. 

Thrombosis  of  the  longitudinal  sinus  generally 
complicates  some  general  debilitating  disease  and  seldom 
arises  from  direct  infection.  It  may  be  met  with  in 
weakened  and  wasted  children,  after  typhoid  fever, 
or  it  may  complicate  severe  forms  of  anaemia. 

The  symptoms  mostly  depend  upon  interference 
with  the  cerebral  cortex,  and  among  them  are  mental 
dullness,  convulsions,  and  possibly  paralysis  if  the 
vascularity  of  the  motor  areas  is  modified.  In  many 
cases  the  symptoms  are  obscured  by  those  of  the  pre- 
viously existing  general  condition. 


CHAPTER  XLII 

APHASIA 

Etiology. — ^Aphasia  is  the  term  applied  to  speech 
defects  of  cortical  origin,  and  it  most  commonly  occurs 
as  the  result  of  a  vascular  disturbance,  e.g.  thrombosis, 
embolism,  or — less  frequently — ^haemorrhage.  Aphasia 
may  also  exist  in  association  with  tumours,  and  its  pre- 
sence may  then  be  of  assistance  in  localizing  the  seat  of 
the  growth. 

Temporary  attacks  in  which  a  patient  has  difficulty 
in  speaking  for  a  short  time  are  not  infrequently  met 
with.  They  are  probably  caused  by  some  passing  vas- 
cular disturbance  of  the  speech  area  of  the  cortex,  or 
they  may  be  caused  by  "  diaschisis  "  (Monakow),  i.e. 
by  a  temporary  loss  of  function  produced  by  the  shock 
of  a  lesion  some  distance  away. 

When  a  lesion  occurs  in  the  course  of  the  motor 
fibres  below  the  level  of  the  cortex,  there  may  be  diffi- 
culty in  speech  due  to  the  inability  of  the  impulses  to 
pass  down  from  the  cortex,  and  in  some  instances  this 
may  amount  to  a  complete  motor  aphasia,  the  power  of 
nnderstanding  remaining  unimpaired. 

The  views  concerning  the  mechanism  connected  mth 
the  functions  of  speech  are  still  in  the  stage  of  con- 
troversy. The  older  and,  until  recently,  generally 
accepted  theories  which  assume  the  presence  of 
"  centres  "  can  most  easily  be  explained  by  considering 
the   education  of  a  child. 

The  first  step  is  the  recognition  of  certain  sounds 
which,  by  repetition,  come  to  be  associated  with  certain 
objects.     Thus  the  memory  for  words  is  first  developed 

411 


412    DISEASES   OF   THE   NERVOUS   SYSTEM 

in  an  area  of  brain  cortex  situated  in  the  1st  temporal 
convolution  of  the  left  side. 

After  a  time  the  child  ceases  to  be  content  to  hear 
words  but  wishes  to  produce  them  also,  and,  after  re- 
peated trials,  succeeds  in  doing  so  by  developing  an 
existing  (inherited)  track  to  a  portion  of  the  cortex 
connected  with  movements  of  the  tongue  and  lips.  This 
part  is  situated  in  the  posterior  part  of  the  3rd  frontal 
convolution  of  the  left  side,  and  is  known  as  Broca's 
convolution.     (Fig.  101.) 

The  child  is  now  able  to  understand  what  is  said 
and  to  make  himself  understood  by  others. 


Fig.  101.— Motor-speech  and  auditory  centres  of  the  brain. 

Further  development  takes  place   by  teaching  the 
child  to  read  and  write. 

First  the  letters  have  to  be  recognized  by  educating 
the  visual  centre  in  the  angular  gyrus,  and  afterwards 
they  have  to  be  reproduced  by  the  education  of  the 
writing  centre,  which  is  situated  (probably)  in  the 
ascending  frontal  convolution  close  to  the  centre  for 
other  movements  of  the  hand  (Fig.  102). 
I  We  have  now  the  two  pairs  of  centres  thus  developed, 
and,  to  make  the  powers  of  communication  still  wider, 
further  education  makes  it  possible  for  impulses  to  pass 
from  the  auditory  to  the  visual  centres,  and  vice  versa, 


APHASIA:    EXAMINATION 


413 


so  that  tlie  child  is  able  to  read  aloud  (in  which  the 
impulses  pass  from  the  visual  to  auditory,  to  Broca's 
convolution)  and  to  write  from  dictation  (in  which 
impulses  pass  from  auditory  to  visual  and  to  writing 
centres)  (Fig.  103). 

In  right-handed  people  those  centres  are,  as  a  rule, 
all  situated  in  the  cortex  of  the  left  hemisphere,  but 
in  those  who  are  left-handed  the  position  is  generally 
reversed. 

Examination. — In  examining  a  case  of  aphasia, 
the  function  of  these  four  centres  and  the  integrity  of 
their  commissural  fibres  should  be  tested  in  turn,  and 


Fig.  102.— Visual  and  writing  centres  of  the  brain. 

the  following  method  is  based  on  the  plan  adopted  by 
C.  E.  Beevor  : — 

1.  Test  the  first  pair  of  centres,  viz.  the  motor  and 
auditory  centres,  and  their  commissural  fibres. 

(a)  The  motor  centre  (S). — Can  the  patient  speak 
intelligently  ?  This  tests  the  condition  of  the  motor 
mechanism  at  Broca's  convolution.  If  this  centre 
is  destroyed  he  loses  the  power  of  coordinate  speech, 
and  is  unable  to  repeat  the  names  of  words  or  objects 
though  he  may  still  be  able  to  say  a  few  words,  usually 
the  same  ones  over  and  over  again.  This  condition 
is  known  as  motor  aphasia. 


414    DISEASES   OF   THE   NERVOUS   SYSTEM 

[b)  The  auditory  receptive  centre  (A). —  Can  the 
patient  understand  what  he  hears  ?  Speak  some  simple 
command,  such  as  "  Stand  up,"  or  "  Hold  up  your 
hand."  This  tests  the  condition  of  the  auditory  recep- 
tive centre.  If  this  centre  is  destroyed,  sounds  lose 
their  meaning  and  convey  no  understanding — a  condition 
spoken  of  as  word-deafness. 

(c)  Communication  between  auditory  and  motor  centres 
(A-S). — Can  the  patient  repeat  names  of  things  or 
letters  that  he  hears  ?  This  tests  the  condition  of  the 
fibres  between  the  auditory  and  the  motor  centres. 


Fig.  103.— Auditoi'y  and  visual  centres  of  the  brain. 

2.  Next  test  the  second  pair,  viz.  the  visual  and 
writing  centres,  and  their  commissural  fibres. 

{a)  The  visual  centre  (V). — Can  the  patient  -under- 
stand ™tten  language  ?  Place  before  him  some  written 
request,  such  as  "  Stand  up,"  or  "  Hold  up  your  hand." 
This  tests  the  visual  centre  in  the  angular  gyrus,  and,  if 
its  function  is  impaired,  the  letters,  though  seen,  convey 
no  intelligible  meaning,  and  the  condition  is  then  spoken 
of  as  word-blindness. 

(6)  The  writing  centre  (W). — Can  the  patient  write 
spontaneously  ?  This  tests  the  centre  for  writing, 
which  is  probably  situated  near  or  in  the  area  for  that 
of  movement  of  the  hand.     Failure  is  known  as  agraphia. 


APHASIA:    EXAMINATION  415 

This  test  is  often  difficult  to  apply — first,  because  the 
auditory  or  visual  centre  has  to  be  intact  in  order  to 
convey  the  command ;  and  secondly,  because  paralysis 
of  the  right  hand,  which  is  so  frequently  associated  with 
aphasia,  makes  writing  impossible.  It  is  only  very 
rarely  that  agraphia  exists  alone. 

(c)  Communication  between  the  visual  and  the  writing 
centre  (V-W).  Test  the  commissural  fibres  from  the 
visual  to  the  writing  centre  by  asking  the  patient  to 
copy  from  printed  to  written  characters,  which  entails 
a  visual  impression  that  has  been  understood  passing 
to  the  centre  for  hand  movements. 

3.  Test  the  communication  between  the  auditory  and 
visual  centres — from  the  auditory  to  visual,  to  writing 
centres  ;  from  the  visual  to  auditory,  to  motor  speech 
centre. 

{a)  Communication  between  auditory  and  visual  centres. 
— Test  the  fibres  which  connect  the  auditory  and  the 
visual  centre  (A-V) ;  for  this  purpose  ask  the  patient  to 
pick  out  letters  or  objects  the  names  of  which  he  hears. 
This  entails  the  impulses  passing  from  the  auditory  to 
the  visual  centres  and  being  comprehended  at  both. 

(6)  Communication  from  auditory  to  visual  and  to 
writing  centres  (A- V-W).  Test  this  by  asking  the 
patient  to  write  from  dictation. 

(c)  Communication  from  visual  to  auditory  and  to 
motor-s'peech  centre  (V-A-S). — Test  this  by  asking  the 
patient  to  read  aloud  and  understand  what  he  is  reading. 

If  these  tests  for  the  different  centres  are  applied 
methodically,  noting  after  each  where  the  patient  succeeds  . 
and  where  he  fails,  some  idea  of  the  extent  of  the  lesion 
will  generally  be  obtained,  but  m  practice  the  investiga- 
tion is  often  made  difficult  by  the  partial  or  complete 
destruction  of  more  than  one  area. 

Individuals  also  vary  considerably  as  regards  the 
effects  of  partial  lesions.  Failure  to  read  (alexia)  especially 
seems  to  vary  in  this  respect,  and  is  no  doubt  often  in- 
fluenced by  the  degree  to  which  the  patient  has  been 
educated.     Where  a  patient  knows  several  languages, 


416    DISEASES   OF   THE   NEKVOUS   SYSTEM 

a  comparison  sometimes  shows  that  he  has  retained 
more  command  over  some  than  over  others. 

Also,  from  subcortical  lesions,  the  impulses  may  be 
prevented  from  reaching  a  centre  though  the  centre 
itself  remains  intact.  If  this  should  occur  in  the  case  of 
the  auditory  centre,  the  patient  may  be  "  word-deaf  " 
though  he  can  still  talk,  since  his  memory  for  words 
exists.  Similarly,  in  the  case  of  the  visual  centre, 
the  patient  may  be  unable  to  recognize  printed  words 
and  yet  be  able  to  express  himself  in  writing. 

It  is,  of  course,  important  not  to  confuse  a  peripheral 
lesion  which  impairs  the  articulation  of  language  with 
aphasia. 

While  the  conception  of  aphasia  as  outlined  above 
has  had  its  uses  as  a  working  hypothesis,  it  gradually 
became  api)arent  that  the  schematic  representations 
were  too  artificial  and  that,  in  practice,  the  symptoms  of 
any  particular  case  can  seldom  be  made  to  correspond 
satisfactorily  with  the  diagrammatic  representations. 
Too  much  stress  had  been  laid  on  the  anatomical  defini- 
tion of  "  centres  "  and,  in  order  to  explain  away  the 
discrepancies,  unsatisfactory  attempts  often  had  to  be 
made  to  bring  the  symptoms  into  correspondence  with 
the  theories.  In  1905  Pierre  Marie  expressed  his  dis- 
satisfaction with  these  stereotyped  views,  and  maintained 
that  the  idea  of  the  existence  of  separate  "  centres  "  for 
the  functions  of  speech  could  no  longer  be  upheld,  and 
that  it  was  erroneous  to  believe  that  impulses  conveying 
the  elements  of  speech  are  fixed  in  special  localities. 
To  Marie,  speech  is  an  intellectual  function  for  the 
adequate  performance  of  which  a  particular  portion  of  the 
brain  is  set  aside,  and  consequently  damage  to  this  par- 
ticular portion  will  produce  more  or  less  failure  of  speech 
as  a  whole  rather  than  any  particular  elements  of  it — 
the  degree,  of  course,  varying  according  to  the  intensity 
of  the  lesion  and  the  education  of  the  individual.  This 
idea  is  supported  by  the  fact  that  in  cases  of  partial 
aphasia  it  is  the  latest  and  highest  acquirements  that 
suffer  first,  just  as  it  is  in  other  branches  of  intellectual  dis- 


APHASIA 


417 


order.  Thus,  a  patient  who  knows  several  languages  may 
lose  the  power  of  expressing  himself  in  those  with  which 
he  is  least  familiar ;  and  likewise,  a  man  who  knows 
only  one  language  will  find  the  greatest  difficulty  of 
expression  with  the  more  complicated  parts  of  it. 

Marie  maintains  that  in  every  case  of  aphasia  due 
to  a  cortical  lesion  there  is  some  degree  of  intellectual 
impairment,  as  shown  by  the  failure  to  comprehend 
spoken  language,  and  that  the  omission  to  observe 
this  fact  hitherto  has  been  due  to  the  too  simple  char- 
acter of  the  tests  that  have  been  applied. 


Fig.  104.— Lesion  of  angular  gyrus  and  bases  of  two  upper  tempora;! 
lobes  accompanied  by  aphasia  of  Wernicke.    [Pierre  Marie.) 

The  area  which  Marie  considers  to  be  the  seat  of  this 
intellectual  process  is  known  as  the  zone  of  Wernicke, 
and  comprises  the  supramarginal  convolution,  the  angu- 
lar gyrus,  and  the  base  of  the  1st  and  2nd  temporal 
convolutions   (Fig.  104). 

The  intellectual  impairment  which,  according  to 
Marie,  is  the  essential  factor  of  cortical  aphasia  may  be 
accompanied  by  loss  of  power  to  speak  if  the  lesion 
extends  into  and  affects  the  conduction  of  the  tracts 
of  the  subcortical  region,  a  loss  of  power  which  he  terms 
anarthria.     Most  striking  of  all,  however,   is  his  view 


418    DISEASES   OF   THE   NERVOUS   SYSTEM 

that  Broca's  convolution  plays  no  part  in  the  production 
of  speech,  which  view  he  supports  by  the  citation  of 
cases  in  which  tumours  and  other  lesions  of  this  area 
have  been  unaccompanied  by  any  signs  of  motor 
aphasia.     (Fig.  105.) 

It  is  not,   of  course,   denied  that  aphasia  is  often 
associated  with  lesions  of  Broca's  convolution,   but  it 


Fig.  105.~Coitica,l  lesiou  nt  the  level  of  the  3rd  left  frontal 
couvolutiou  in  a  right-handed  man,  Avithout  any  dis- 
turbance of  speech.     (Pierre  Marie) 

is  maintained  that  a  close  examination  of  such  instances 
shows  that  the  destruction  of  tissue  is  not  strictly  limited 
to  this  area. 

The  three  terms,  Wernicke's  aphasia,  Broca's  aphasia, 
and  anarthria,  are  described  by  Marie  as  follows  : 

(1)  In  the  aphasia  of  Wernicke  the  patients  can  speak, 
but  their  intellect  is  always  impaired  to  a  variable 
extent,  so  that  they  may  talk  nonsense  ;  there  is  also 
loss  of  the  power  of  reading  and  writing,  and  an  incapa- 
bility of  comprehending  spoken  language. 


APHASIA  419 

(2)  In  the  aphasia  of  Broca  the  patients  can  neither 
read  nor  write,  neither  can  they  comprehend  fully  all 
that  is  said  to  them.  So  far,  they  resemble  the  cases 
of  aphasia  of  Wernicke,  but  they  have  the  additional 
symptoms  of  being  unable  to  speak. 

(3)  In  anarthria  there  is  simply  an  inability  to 
speak  from  interruption  of  the  motor  fibres  below  the 
cortex.  This  disability  is  met  with  in  association  with 
lesions  of  the  lenticular  zone ;  and  Marie  maintains 
that  the  aphasia  of  Broca  consists  of  the  aphasia  of 
Wernicke  together  with  anarthria,  and  that  it  is  not  in 
any  way  connected  with  lesions  of  the  third  left  frontal 
convolution. 

And  thus  it  comes  about,  as  stated  above,  that  Marie 
recognizes  only  one  form  of  aphasia,  viz.  the  aphasia 
of  Wernicke,  which  he  localizes  in  the  supramarginal 
convolution,  the  angular  gyrus.  While  Marie's  views  met 
with  considerable  opposition,  more  especially  with  regard 
to  the  exclusion  of  Broca 's  convolution,  it  was  generally 
felt  that  an  important  advance  had  been  made  by  raising 
the  question  and  by  suggesting  a  possible  outlet  from  a 
position  which  was  frequently  difficult  to  reconcile  with 
clinical  facts. 

Still  more  recently  the  subject  has  been  entirely 
reopened  by  Head,"^  who  has  followed  up  suggestions 
originally  made  by  Hughlings  Jackson.  As  the  result 
of  elaborate  investigations  Head  concludes  that  dis- 
orders in  the  use  of  language  due  to  a  unilateral  cortical 
lesion  cannot  be  ascribed  to  destruction  of  isolated 
'"'  centres."  He  insists  that  in  such  disorders  there  is 
interference  with  the  use  of  language  as  a  whole,  and 
though  certain  aspects  of  it  may  be  disturbed  more  than 
others,  according  to  the  situation  of  the  lesion,  the  defects 
of  speech  so  produced  cannot  be  expressed  in  simple 
terms  of  audition,  articulation,  vision,  and  writing. 
Head's  view  is  that  a  cortical  lesion  causes  dissociation 
of  the  highest  forms  of  expression  of  thought  (i.e.  of 
symbolic  thinking  and  expression),  and  according  to 
*  £niw,  vol,  xliif,,  pt.  ii,,  1920. 


420    DISEASES   OF   THE   NERVOUS   SYSTEM 

the  principal  dissociations  that  have  been  observed  to 
occur  he  has  suggested  the  following  provisional 
classification  : 

(a)  Verbal  ajjhasia,  which  is  essentially  a  defect 

of  word  formation. 
(6)  Nominal  aphasia,  characterized  by  defective 
use  of  names  and  want  of  comprehension  of 
the  nominal  value  of  words  or  other  symbols. 

(c)  Sy7itactical  ajjhasia,  in  which  the  patient  tends 

to  talk  jargon. 

[d)  Semantic   aphasia    {o-iqiiaiveLv^   to   signify),  in 

which  there  is  a  want  of  recognition  of  the 
full  significance  of  words  and  phrases.     The 
patient  may  understand  each  word  or  short 
phrase  exactly  as  he  can  comprehend  the 
details  of  a  picture,  but  the  ultimate  meaning 
escapes  him. 
In  most  cases  of  aphasia  two  or  more  of  these  aspects 
of  symbolic  formation  are  affected,  and  in  order  to  under- 
stand  the   nature   of   the   disordered   functions   Head 
advises    that    examination    should    first    be    made    of 
patients  in  whom  the  disturbance  is  as  nearly  as  possible 
confined  to  one  of  these  forms,  after  which  the  more 
complicated  cases  will  be  the  better  understood. 

Treatment. — When  there  is  reason  to  think  that 
the  aphasia  is  due  to  pressure  on  the  cortical  area  for 
speech  by  tumours,  extradural  hsemorrhages,  or  other 
lesions  of  a  removable  character,  it  may  be  possible  to 
obtain  relief  by  a  surgical  operation. 

When  the  functions  of  the  centres  have  been  per- 
manently impaired,  treatment  should  be  directed  towards 
re-educating  the  patient,  by  which  means  improvement, 
at  any  rate  to  some  degree,  may  frequently  be  obtained. 
The  chances  of  improvement  are  greatest  in  children, 
provided  always  that  sufficient  intelligence  exists  to 
make  them  capable  of  learning,  and  it  is  thought 
that  possibly  in  some  such  cases  the  right  hemisphere 
of  the  brain  takes  up  the  function  after  that  for  the 
left  has  been  destroyed. 


CHAPTER  XLIII 

MYASTHENIA  GRAVIS  (ASTHENIC   BULBAR 
PARALYSIS) 

The  characteristic  symptom  of  myasthenia  gravis  is 
the  ease  with  which  muscles  become  exhausted.  Partial 
recovery  of  power  often  occurs  after  a  period  of  rest, 
but  the  affected  muscles  are,  as  a  rule,  incapable  of 
carrying  out  a  sustained  movement  for  any  length  of 
time. 

Etiology  and  pathology. — The  sexes  suffer  in 
about  equal  proportions,  and  in  most  cases  the  symptoms 
occur  during  the  first  half  of  life.  There  is  still  a  consider- 
able degree  of  doubt  concerning  the  morbid  conditions 
which  underhe  the  symptoms. 

So  far  as  the  central  nervous  system  is  concerned, 
beyond  the  occasional  observance  of  some  indefinite 
and  inconstant  phanges  in  the  nuclei  of  the  cranial  nerves, 
nothing  notable  has  been  recorded. 

General  opinion  at  the  present  time  favours  the 
view  that  the  essential  changes  occur  in  the  muscles 
themselves,  and  in  support  of  this  hypothesis  is  the 
finding  by  Weigert  of  an  exudation  of  lymphoid  cells  in 
the  muscle  tissue,  an  observation  which  has  been  con- 
firmed by  Hun,  G-oldflam,  Farquhar  Buzzard,  and  others. 
It  was  present  also  in  one  of  the  cases  which  the  author 
had  an  opportunity  of  examining. 

Another  abnormality  often  found  is  an  enlarged  thy- 
mus gland  (Fig.  106)  which  is  sometimes  of  a  lympho- 
sarcomatous  character,  but  the  connection,  if  any,  exist- 
ing between  this  and  the  exudation  of  lymphoid  cells 

421 


422    DISEASES   OF   THE   NERVOUS   SYSTEM 

into  the  muscles  is  uncertain.  Changes  in  the  supra- 
renal bodies  have  also  been  described.  At  present  one 
must  be  content  to  note  the  lesions  and  to  assume  that 
the  disease  depends  either  upon  some  modification  of 
the  internal  secretions  or  upon  some  toxin  of  which  the 
origin  and  composition  are  as  yet  unknown. 

Symptoms. — The    symptoms    are    brought    about 
by  failure  of  muscular  power,  but,  as  this  is  unequally 


g|uu|^^|uuuuuyum||iuuyuuuuyu|iy|iuuyyu^ 


Fig.  106.  —  Eularged   thymus   gland   in    a    case  of 
myasthenia  gravis. 

distributed,  they  vary  in  different  cases  according  to 
the  parts  affected. 

There  are  also  great  fluctuations  in  the  intensity 
of  the  symptoms,  alternate  exacerbations  and  remissions 
being  very  characteristic  of  the  disease.  The  "  ocular  " 
and  "  bulbar  "  distributions  are  common. 

In  the  case  of  the  ocular  symptoms,  ptosis  is  generally 
a  prominent  feature  (Fig.  107),  and  the  patients  com- 
plain that  the  eyes  cannot  be  kept  open  for  long  at 
a  time.  The  lids  persistently  droop,  and  attempts  are 
frequently  made  to  correct  the  inconvenience  by  throw- 
ing back  the  head  or  even  holding  up  the  eyelids  with 
the  fingers.     A  night's  rest  often  parti'ally  restores  the 


MYASTHENIA    GKAVIS :    SYMPTOMS       423 

power,  which  again  wanes  as  the  day  wears  on,  and 
any  sustained  movement,  such  as  looking  upwards  for 
any  length  of  time,  rapidly  exhausts  the  muscles  and 
brings  on  the  drooping. 

The  compensatory  action  of  the  occipito-frontalis, 
seen  in  the  wrinkling  cf  the  brow,  which  is  a  usual 
feature  of  ptosis  from  other  causes,  is  generally  absent, 


Fig.  107.— Bilateral  ptosis  in  myasthenia  gravis. 

since  the  muscle  is  quickly  exhausted  by  any  attempts 
at  continuous  contraction.     (Fig.  108.) 

Transient  ocular  palsies  of  varying  nature  are  com- 
mon, and  occasionally  nystagmus  is  present. 

The  bulbar  symptoms  appear  chiefly  in  connection 
with  the  orbicularis  oris  and  the  muscles  of  the  tongue 
and  palate. 

Weakness  of  the  orbicularis  oris  makes  it  difiicult 
to  blow  or  whistle,  and  also  afiects  the  speech. 


rzi    DISEASES   OF  THE  NERVOUS  SYSTEM 

The  tongue  when  exhausted  cannot  be  properly- 
thrust  out  or  forcibly  protruded  into  the  cheek.  The 
paralysed  soft  palate  ceases  to  shut  off  the  nasal  from 
the  oral  cavity,  and  so  the  nasal  voice  is  produced, 
and  at  the  same  time  there  may  be  difficulty  in  swallowing, 
with  regurgitation  of  fluids  through  the  nose.  All  these 
muscles  become  weaker  after  use,  and  exhaustion  of 
the  masseters,   w^hich  often  takes  place  during  masti- 


Fig.  1C8.— Ptosis  and  general  facial  expres- 
sion   in   myasthenia  gravis.    Note  the 
absence  of   any   compensatory  action 
of  the  occipito-froutaiis.    (Bij  cuioiesy 
of  Dr.  George  Johnston.) 

cation,  may  make  it  impossible  to  finish  a  meal.  The 
facial  muscles  may  also  suffer,  and  the  weakness  of  the 
muscles  round  the  mouth  often  causes  an  alteration  in 
the  expression  which  closely  simulates  that  associated 
with  the  myopathies. 

Another  characteristic  is  the  "  nasal  smile,"  in 
which  the  movement  at  the  corners  of  the  mouth  is 
deficient,  the  furrow  of  the  smile  being  sometimes 
entirely  above  the  upper  fip  (Gowers). 


MYASTHENIA    GRAVIS  425 

When  the  limbs  are  at  fault,  walking  soon  causes 
fatigue,  and  no  occupation  involving  the  use  of  the 
arms  can  be  engaged  in  for  any  length  of  time.  In 
the  trunk,  the  muscles  of  the  neck  often  suffer  and 
the  head  falls  forward  on  the  chest.  Weakness  of  the 
muscles  of  respiration  causes  dangerous  attacks  of 
dyspnoea. 

Electrical  reactions. — The  rapidity  with  which  the 
power  of  the  muscles  is  exhausted  by  repeated  con- 
tractions can  be  demonstrated  by  stimulating  them 
with  the  faradic  current.  After  a  variable  number  of 
contractions  have  been  induced,  the  response  becomes 
gradually  weaker  and  weaker,  and  finally  ceases  alto- 
gether, but  may  again  be  renewed  after  a  period  of  rest. 
After  the  muscle  has  ceased  to  respond  to  the  electrical 
stimulus  it  may  still  be  capable  of  voluntary  contrac- 
tions. This  condition  is  known  as  the  myasthenic 
reaction,  and  when  present  is  very  characteristic  of  the 
disease ;  but,  like  the  reaction  of  the  muscles  from 
voluntary  movements,  it  varies  from  time  to  time, 
nor  is  it  necessarily  equally  obvious  in  all  the  affected 
muscles  at  one  time. 

Reflexes. — The  reflexes  differ  in  their  reactions 
according  to  the  condition  of  the  muscles  which  take 
part  in  their  production.  The  knee-jerks  are  some- 
times absent,  or  they  can  be  exhausted  by  repeatedly 
eliciting  them.  Wasting  of  muscles  is  uncommon,  but 
has  occasionally  been  observed. 

Sensory  changes. — While  the  main  symptoms  are 
motor,  there  are  occasionally  some  sensory  changes  to 
be  noted.  Aches  and  pains  are  liable  to  occur,  and 
Farquhar  Buzzard*  has  recorded  cases  in  which  the 
pains  closely  resembled  in  their  intensity  those  of  loco- 
motor ataxy.  The  same  observer  has  also  shown  that 
analgesia  and  anaesthesia  may  be  found,  and  that  the 
presence  of  mental  symptoms  is  not  unknown. 

Diaguosls. — For  purposes  of  diagnosis  the  cases 
of  myasthenia  gravis  may  be  divided  into  three  groups, 
*  Brain,  Part  cxi. 


426    DISEASES   OF   THE   NERVOUS   SYSTEM 

viz.  ocular,  bulbar,  and  spinal,  according  to  the  parts 
in  which  the  early  symptoms  predominate. 

1.  Ocular  types. — When  ocular  symptoms  are  the  pro- 
minent feature,  other  conditions,  in  which  ptosis  and 
partial  ophthalmoplegia  externa  are  likely  to  occur, 
must  be  considered.  In  tabes  when  these  symptoms 
are  present  there  are  almost  certainly  other  distinc- 
tive signs,  such  as  an  Argyll-Robertson  pupil,  in  asso- 
ciation with  lightning  pains,  loss  of  knee-jerks,  and 
incoordination. 

Transient  ocular  symptoms  are  sometimes  associated 
with  migraine,  but  here  they  generally  bear  some  definite 
relation  to  the  headache. 

Cerebral  tumours,  syphilitic  disease,  and  hysteria  must 
also  be  considered  as  causes  of  ptosis.  In  hysteria 
the  ptosis  is  usually  spasmodic  rather  than  paralytic, 
but  outwardly  it  may  show  some  similarity  to  that  of 
myasthenia  by  the  absence  of  the  compensatory  furrows 
of  the  brow. 

2.  Bulbar  type. — When  signs  of  bulbar  trouble  are 
prominent,  progressive  bulbar  paralysis  and  post-diph- 
theritic neuritis  will  suggest  themselves  ;  in  both  these 
conditions  the  distribution  of  symptoms  is  apt  to  be 
similar.  In  progressive  bulbar  paralysis  the  early  and 
pronounced  wasting,  with  no  remissions  and  with  absence 
of  the  myasthenic  reaction,  will  serve  to  enable  the 
distinction  to  be  made. 

In  postdiphtheritic  neuritis  the  history  and  course 
are  usually  distinctive,  and  here  again  the  electrical 
reactions  will  assist  in  the  diagnosis.  In  very  rare 
instances  the  diphtheria  may  cause  permanent  palsy 
of  a  bulbar  type,  cases  of  which  have  been  recorded  by 
Wilfred  Harris.*  The  distribution  of  the  paralysis  in 
these  cases  very  closely  resembles  that  met  with  in 
myasthenia  gravis,  but,  as  Harris  points  out,  the  affec- 
tion is  usually  limited  to  one  or  more  of  the  bulbar 
nuclei,  so  that  ptosis,  and  weakness  of  muscles  of  the  jaw 
and  of  the  neck  and  limbs,  are  not  present,  as  they  so 
*  Jirain,  Part  civ. 


MYASTHENIA    GRAVIS  427 

often  arc  in  myasthenia.  Moreover,  there  will  be  no 
variation  of  symptoms  and  no  myasthenic  reaction, 
while,  on  the  other  hand,  there  will  be  wasting  and 
reaction  of  degeneration. 

3,  Spinal  type. — Where  vague  symptoms  of  spinal 
distribution  are  complained  of,  such  as  fatigue  after  slight 
exertion,  an  erroneous  diagnosis  of  neurasthenia  may 
easily  be  made. 

Prognosis. — The  outlook  in  the  majority  of  cases 
is,  in  the  long  run,  bad,  though  remissions  and  inter- 
missions of  long  duration  may  occur,  and  in  a  small 
percentage  of  cases  the  symptoms  seem  to  have  per- 
manently disappeared.  The  tendency  is  for  the  patient 
to  become  more  and  more  easily  exhausted,  and  the 
danger  to  life  lies  chiefly  in  the  occurrence  of  attacks 
of  cardiac  failure  and  respiratory  dyspnoea. 

Treatment. — ^In  the  absence  of  a  definite  knowledge 
of  the  cause  of  the  trouble,  efforts  have  to  be  directed 
towards  preserving  the  contractility  of  the  muscles.  Rest, 
with  the  administration  of  strychnine  and  other  tonics, 
is  at  present  the  best  that  can  be  done.  The  frequency 
with  which  an  enlarged  thymus  gland  has  been  found 
suggests  the  possibility  of  some  modification  or  absence 
of  an  internal  secretion  as  the  cause  of  the  disease,  but 
hitherto  the  administration  of  glandular  preparations 
has  not  been  followed  by  any  definite  results 


CHAPTER   XLIV    • 

CONVULSIONS 

Ir  frcqaently  happens  that  a  patient  is  seen  for  the 
first  time  on  account  ot  "  fits."  It  is  therefore  im- 
pDrtant  to  review  the  general  subject  of  convulsions, 
which  outwardly  may  give  no  clue  to  their  different 
causes. 

It  is  convenient  to  divide  the  subject  into  two  parts, 
viz.  convulsions  as  they  occur  (1)  in  childhood  and  (2) 
in  adults. 

I.  Convulsions  in  childliood. — Some  of  the 
principal  conditions  which  are  associated  with  convul- 
sions during  childhood  are :  rickets,  idiopathic  epilepsy, 
infantile  hemiplegia  and  diplegia  from  various  causes, 
meningitis,  and  cerebral  tumours. 

The  commonest  condition  which  underlies  the  con- 
vulsions of  children  is  rickets. 

The  well-known  tendency  to  convulsions  in  children  is 
considered  to  be  primarily  due  to  an  incomplete  develop- 
ment of  the  central  nervous  system,  so  that  the  different 
parts  are  not  under  adequate  control.  Thus  a  healthy 
infant  is  always  more  liable  to  convulsions  than  a  healthy 
adult,  but  should,  nevertheless,  if  properly  cared  for, 
possess  a  nervous  system  sufficiently  stable  to  enable 
him  to  pass  safely  through  this  period  of  life,  unless 
exposed  to  some  unusually  severe  strain,  as,  for  example, 
the  incidence  of  an  acute  specific  fever.  If,  however, 
the  naturally  incomplete  development  is  further  re- 
tarded by  neglect,  rickets,  or  other  circumstances,  the 
stability  will  be  still  further  lowered  in  varying  degrees, 

428 


CONVULSIONS  429 

and  in  that  case  comparatively  slight  variations  in 
general  health — ^intestinal  disturbances,  or  other  forms 
of  irritation — suffice  to  bring  on  a  fit. 

Three  types  of  children  can  be  recognized  in  this 
respect  : 

(1)  The  healthy  infant,  in  whom  the  relationship 
between  different  parts  of  the  nervous  system  has  not 
yet  fully  developed,  but  who  still  possesses  a  nervous 
system  sufficiently  stable  under  all  ordinary  circum- 
stances. 

(2)  The  child  whose  development  has  been  abnor- 
mally retarded.  Here,  with  care,  convulsions  may  just 
be  avoided,  but  the  nervous  system  in  this  case  is  very 
lightly  balanced,  and  a  fit  will  readily  occur  from  local 
or  general  disturbances. 

(3)  The  child  whose  nervous  system  is  so  unstable 
that  convulsions  occur  in  spite  of  all  care  to  avoid  undue 
stimulation. 

As  the  stability  of  the  nervous  system  increases 
with  the  growth  of  the  child  the  convulsions  often 
cease,  but  in  a  certain  number  of  cases  this  desired 
stability  is  never  attained,  and  the  child  continues  to 
be  "subject  to  fits."  It  is  this  class  which  forms  an 
important  link  between  the  convulsions  of  childhood 
and  the  epilepsy  of  adults,  and  between  the  two  every 
gradation  may  be  traced. 

A  recognition  of  these  groups  makes  the  part  played 
by  teething,  constipation,  flatulence,  and  other  kinds 
of  irritation  in  the  causation  of  the  fits  easily  under- 
stood. A  healthy  child  may  pass  successfully  through 
them  all,  but  they  will  prove  too  strong  for  the  possessor 
of  an  abnormally  unstable  nervous  system  and  will 
start  the  impulses  which  give  rise  to  the  fit. 

The  convulsions  connected  with  the  other  cerebral 
conditions  mentioned  above  are  incidents  in  the  courses 
of  the  diseases  and  do  not  require  separate  description. 

2.  Convulsions  of  adults  are  chiefly  associated 
with  epilepsy,  toxaemias  (e.g.  uraemia,  chronic  alcohoHsm, 
and  poisons  such  as  strychnine),  degenerations  (e.g.  in 


430    DISEASES   OF   THE   NERVOUS   SYSTEM 

general  paralysis  of  the  insane),  meningeal  inflammations, 
cerebral  tumours,  hysteria,  and  onset  of  vascular  lesions 
(haemorrhages,  thrombosis,   embolism). 

Reference  to  the  chapter  on  Epilepsy  (Chapter  XLV.) 
shows  that  first  attacks  of  this  disorder  become  rarer 
as  age  advances,  and  therefore,  when  convulsions  occur 
for  the  first  time  after  middle  life,  suspicions  should 
be  entertained  of  the  possibility  of  cerebral  degeneration 
(especially  general  paralysis  of  the  insane),  cerebral 
tumours,  and  ursemia.  It  must,  however,  be  remem- 
bered that  patients  who  have  taken  a  great  deal  of 
alcohol  sometimes  about  this  age  have  epileptiform  fits 
which  do  not  necessarily  seem  to  signify  the  onset  of  any 
immediate  cerebral  degeneration. 

In  tumours  of  the  brain  a  general  convulsion,  quite 
apart  from  the  localized  Jacksonian  fit,  is  sometimes 
an  early  symptom,  and  may  for  a  time  be  regarded  as 
idiopathic  epilepsy.  It  is  therefore  important  to  examine 
the  discs  of  all  patients  who  complain  of  fits  of  recent 
origin,  even  though  it  appears  from  absence  of  other 
symptoms  that  the  fits  are  due  to  epilepsy. 

The  fits  of  the  general  paralytic  may  likewise  be  an 
early  symptom  of  that  disease,  although  they  more 
often  occur  when  the  nature  of  the  disease  can  be  recog- 
nized from  other  signs.  As  far  as  the  actual  attack 
is  concerned,  it  resembles  that  of  an  epileptic  fit,  either 
generalized  or  of  a  localized  "  Jacksonian  "  character ; 
but  whereas  the  ordinary  epileptic  generally  shows  no 
particular  after-effects  from  the  attacks,  in  the  general 
paralytic  every  fit  tends  to  leave  the  patient  worse,  both 
mentally  and  jjhysically,  than  he  was  before.  Some- 
times apoplectiform  fits,  closely  resembling  those  of 
cerebral  haemorrhage,  alternate  with  the  epileptiform 
seizures,  which  does  not  happen  in  ordinary  epilepsy. 
It  is  thought  that  the  fits  in  these  cases  are  induced 
by  the  irritative  properties  of  the  products  of  the  de- 
generation of  nervous  tissue  taking  place  in  the  cerebral 
cortex.  It  is  also  probable  that  fits  may  sometimes 
be  produced  by  a  localized  parenchymatous  syphilitic 


CONVULSIONS    IN   ADULTS  431 

degeneration  which  does  not  necessarily  extend  or  lead 
on  to  general  paralysis. 

Uraemic  convulsions  occur  occasionally  as  isolated 
symptoms,  but  are  generally  accompanied  by  definite 
signs  of  advanced  renal  disease.  Sometimes  the  symp- 
toms resemble  those  of  cerebral  haemorrhage  more  than 
those  of  epilepsy. 

Convulsions  may  occur  in  the  initial  stages  of  vas- 
cular lesions  of  the  brain  (haemorrhage,  thrombosis,  and 
embolism),  but  the  appearance  of  paralysis  generally 
makes  their  origin  clear. 

Hysterical  fits  are  described  elsewhere  {see  p.  i^O). 


CHAPTER  XLV 

EPILEPSY 

An  attack  of  epilepsy  is  characterized  by  a  sudden  and 
uncontrolled  discharge  of  nerve  energy  which  may  give 
rise  to  all  degrees  of  disturbance,  from  a  momentary 
loss  of  senses  or  a  transitory  attack  of  giddiness  to  a 
severe  convulsion.  The  negative  results  of  an  attack 
are  seen  in  loss  of  consciousness  (partial  or  complete), 
while  the  positive  effects  are  shown  in  convulsive 
movements. 

When  all  the  cases  of  symptomatic  epilepsy  depend- 
ing upon  definite  structural  changes  in  the  brain  have  as 
far  as  possible  been  excluded,  there  still  remains  a  large 
group  for  which  no  adequate  physical  cause  can  be 
found  and  which  are  comprised  under  the  heading  of 
"  idiopathic  "  or  "  essential  "  epilepsy. 

Etiology. — Little  is  definitely  known  about  the 
causation  of  "  essential  "  epilepsy.  In  the  majority  of 
the  cases  the  first  attack  occurs  during  youth,  and  where 
the  fits  seem  to  have  begun  in  later  life  a  careful  in- 
quiry will  often  reveal  the  previous  occurrence  of  minor 
attacks  which  had  passed  almost  unnoticed. 

Convulsions  during  infancy,  whatever  be  their  cause, 
seem  to  dispose  to  epilepsy  in  later  Ufe,  but  there  is 
often  a  considerable  interval  of  freedom.  Especial  care, 
therefore,  should  be  taken  of  children  about  the  age  of 
puberty,  if  they  are  known  to  have  suffered  from  con- 
vulsions in  infancy. 

Epilepsy  tends  to  run  in  neurotic  families,  in  many 
of  which  there  are  also  some  members  mentally  afflicted. 

432 


EPILEPSY:    ETIOLOGY  433 

In  a  person  subject  to  fits  any  undue  peripheral 
irritation  may  turn  the  scale,  and  in  this  way  errors  of 
refraction,  nasal  polypi,  adenoids,  ovarian  and  uterine 
disease,  and  other  sources  of  irritation  may,  in 
certain  circumstances,  serve  to  start  the  explosion.  In 
women  there  is  occasionally  some  relation  between  the 
attacks  and  the  menstrual  periods. 

Of  recent  years  particular  attention  has  been  paid  to 
psychological  factors  in  the  causation,  and  Pierce  Clark 
attributes  the  underlying  state  to  a  congenital  defect  in 
the  general  make-up  of  the  epileptic  mind,  with  which 
are  associated  certain  abnormal  reactions,  e.g.  fits, 
disturbances  of  temper,  lethargies  and  various  psychic 
symptoms.  Assuming  the  existence  of  such  a  primary 
defect,  it  can  be  understood  how  attacks  might  be 
precipitated  by  various  stresses  of  life,  more  particularly 
at  those  periods  when  special  demand  is  made  upon  the 
stability  of  the  nervous  system. 

A  consideration  of  convulsions  in  general  seems  to 
indicate  that  there  are  probably  different  groups  of  cases. 
In  some  it  may  be  that  poisons  arising  within  the  body 
may  be  powerful  enough  to  cause  convulsions  (e.g.  those 
associated  with  uraemia),  and  it  is  not  uncommon  to 
meet  with  convulsions  indistinguishable  from  epilepsy 
as  the  result  of  chronic  alcoholism.  In  such  cases,  how- 
ever, the  stability  of  the  nerve  cells  has  probably  been 
previously  lowered  through  structural  changes,  so  that 
the  fit  is  in  reality  symptomatic  of  nerve  degeneration. 
In  many  cases  it  seems  almost  certain  that  the  fault  of 
the  nervous  system  is  chiefly  local,  as  shown  by  the 
constancy  and  characters  of  the  warning  sensations  at 
the  beginning  of  the  attacks,  the  spreading  of  which  can 
be  traced  from  one  part  of  the  cerebral  cortex  to  another. 
Injuries  to  the  brain,  whether  general  or  local,  whether 
at  birth  or  later  in  life,  may  be  followed  by  fits  which 
clinically  are  indistinguishable  from  those  of  idiopathic 
epilepsy.  Abnormalities  of  internal  secretions,  e.g.  of 
the  thyroid  and  pituitary  glands,  are  thought  by  some 
to  be  factors  in  causing  epilepsy  in  certain  cases,  but  the 

2(; 


434    DISEASES   OF   THE   NERVOUS   SYSTEM 

amount  of  influence,  if  any,  which  they  exert  is  at  present 
quite  uncertain. 

The  mechanism  by  which  the  fit  is  produced  is  also 
uncertain.  The  main  factors  aj)pear  to  be  loss  of  control 
of  the  highest  functional  levels,  accompanied  by  a 
discharge  of  nervous  energy  giving  rise  to  the  convulsion. 
That  the  one  part  may  occur  without  the  other  is  seen 
in  "  minor  "  attacks,  where  the  highest  levels  of  con- 
sciousness are  often  lost  without  the  occurrence  of 
any  accompanying  convulsive  movements.  There 
seems  to  be  no  doubt  that  the  cerebral  cortex  is 
largely  concerned  in  the  mechanism  of  the  attacks, 
as  shown  by  the  nature  of  the  aura  {see  p.  436), 
but  to  describe  the  fits  as  being  due  to  a  "  discharge 
from  the  cerebral  cortex  "  is  a  crude  statement  which 
obviously  requires  qualification. 

At  present  our  knowledge  may  be  summed  up  in  the 
statement  that  an  epileptic  fit  depends  upon  some  want 
of  adjustment  between  the  functions  of  the  cerebral 
cortex  and  the  rest  of  the  nervous  system.  In  some 
cases,  probably  in  many,  it  is  the  nerve  cells  that  are  at 
fault,  so  that  toxins  and  stimuli  are  capable  of  producing 
a  discharge  quite  disproportionate  to  their  intensity  ; 
while  in  others  the  nerve  cells  may  be  of  average  sta- 
bility, but  the  toxins  reaching  them  may  be  too  power- 
ful to  be  withstood,  just  as  the  most  stable  of  them 
will  succumb  to  an  over-dose  of  strychnine  or  to  the 
poison  of  tetanus. 

Syinptoms. — Whether  the  cause  is  of  local  or  of 
general  origin,  the  main  characteristic  of  an  epileptic 
attack  may  be  considered  as  a  discharge  of  nervous 
energy  as  the  result  of  a  disturbance  of  function  of  the 
cerebral  cortex.  Hence  the  symptoms  vary  according  to 
(1)  the  part  in  which  the  disturbance  originates,  (2)  the 
direction  in  which  it  extends,  and  (3)  the  rapidity  with 
which  it  spreads. 

If  a  general  view  of  the  cerebral  cortex  is  taken, 
it  will  be  understood  why  warnings  occur  in  some  cases 
and  not  in  others  :  how  in  some  the  disturbance  of  func- 


EPILEPSY:    SYMPTOxAIS  435 

tion  is  limited  to  a  restricted  area,  and  finally  how  in 
others  a  generalized  convulsion  results. 

When  the  disturbance  starts  in  any  of  the  areas  the 
functions  of  which  are  known,  its  first  effects  are  those 
of  stimulation  of  the  given  area.  Thus  a  discharge 
beginning  in  the  occipital  lobe  will  give  rise  to  visual 
symptoms,  and  in  the  temporo- sphenoidal  lohe  to  auditory 
symptoms,  and  so  on.  If  the  fit  begins  in  the  7notor 
area,  twitching  of  a  limb  is  often  the  first  sign,  just  as 
it  is  when  a  gross  lesion  causes  the  irritation. 

Thus  the  nature  and  position  of  the  aui"a,  which 
may  be  referred  to  almost  any  part  of  the  body,  indicate 
that  j^articular  area  of  the  brain  at  which  the  disturb- 
ance of  function  has  originated. 

The  difi"erent  stages  of  a  fit  may  be  tabulated 
thus : 

1.  Aura. 

2.  Convulsions  : 

{a)  Tonic.     (6)  Clonic. 

3.  After-effects  : 

Sleep,  headache,  and  aching  of  limbs. 

Coma. 

Automatism. 

Temporary  mania. 

Temporary  paralysis. 
At  the  termination  or  in  the  absence  of  the  aura,  the 
patient  suddenly  loses  consciousness,  and,  with  a  respira- 
tory spasm  which  often  produces  a  groan  or  cry  (the 
epileptic  cry),  falls  to  the  ground  with  the  muscles  in  a 
state  of  tonic  contraction.  In  about  half  a  minute  this 
stage  is  succeeded  by  clonic  contractions,  which  last  for 
two  or  three  minutes  or  so  and  then  gradually  pass  off, 
leaving  the  patient  usually  in  a  deep  sleep,  or  leading 
to  one  or  other  of  the  postepileptic  conditions  mentioned 
above. 

The  commonest  after-effect  is  sleep  of  varying  length, 
from  which  the  patient  is  apt  to  wake  with  tired  and 
aching  limbs,  as  though  he  had  been  bruised  all  over. 
Sometimes  the  sleep  deepens  into  comaj 


436    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  aurae. — These  may  be  classified  as  sensory  and 
motor. 

1.  Sensory  aurae. 

(a)  Special  senses  : 

Visual. 
Auditory. 
Smell. 
Taste; 

(b)  General : 

Sensations  of  something  passing  up  a  limb. 
Tingling. 
Giddiness. 
Palpitation. 

Flatulence   and   sensation  in  the   pit  of 
the  stomach   (epigastric  aura). 

(c)  Psychical,  e.g.  dreamy  states,  reminiscences. 

AurcB  of  special  senses. — Of  these  the  visual  and 
auditory  are  common,  while  warnings  of  taste  and  smell 
are  rare.  Visual  aurae  often  take  the  form  of  flashes 
of  light,  partial  or  complete  loss  of  vision,  and  images 
of  various  kinds,  while  loud  noises  and  whistlings  are 
most  frequently  met  with  in  connection  with  the  auditory 
warnings.  Sometimes  words  are  heard,  or  a  part  of  a 
sentence  which  may  always  stop  abruptly  at  the  same 
place  in  successive  attacks,  when  consciousness  is  lost. 
Warnings  of  taste  and  smell  seem  always  to  be  unpleas- 
ant ;  an  indescribable  "  sulphurous  "  smell  or  a  taste 
of  "  rotten  cabbage  "  are  among  those  described. 

AurcB  of  general  sensations. — The  best-known  is  the 
sensation  of  "  something  passing  up  the  arm  or  leg," 
from  which  the  term  "  aura  "  was  originally  derived. 
The  feeling  generally  begins  in  the  hand  and  passes 
deliberately  up  the  arm  until  it  reaches  a  certain  point, 
when  consciousness  is  lost.  With  this  kind  of  aura 
there  is  a  good  chance  of  arresting  the  fit  by  tying  a 
cord  round  the  arm  and  pulling  it  tight  the  moment 
the  sensation  is  felt — a  practical  device  which  has  been 
handed  down  from  ancient  times. 


EPILEPSY:    AVRM  437 

Most  of  these  general  sensations  are  vague  and  call  for 
no  special  description,  and  in  many  of  tliem  the  patients 
find  it  difficult  to  express  their  feelings  in  words. 

Aurce  of  psychical  sensations. — These  warnings  are 
interesting,  and  include  various  dreamy  conditions 
which  are  often  not  altogether  unpleasant.  There  also 
oocur  the  pecuHar  sensations  known  as  "  reminiscences," 
in  which  there  is  a  feeling  of  familiarity  with  a  scene,  as 
though  it  had  all  been  seen  before,  though  it  is  often 
impossible  that  there  can  have  been  any  previous  know- 
ledge of  it.  These  sensations  may  be  experienced  by 
healthy  people,  and  have  been  well  described  by  Dickens, 
Scott,  Tennyson,  Ohver  Wendell  Holmes,  and  other 
writers ;  but  when  they  occur  very  frequently  and 
vividly  they  should  be  regarded  with  suspicion,  and 
careful  inquiry  should  be  made  for  any  other  symptoms, 
such  as  attacks  of  momentary  dizziness,  or  the  signs  of 
spasm  in  the  jaws,  face,  or  eye  muscles,  which,  if  present, 
will  indicate  the  need  for  treatment. 

As  a  rule  an  aura  lasts  from  a  few  seconds  up  to  a 
minute  or  so,  but  sometimes  it  is  of  considerably  longer 
duration,  and  enables  the  patient  to  try  to  check  the 
fit  and  to  get  into  a  place  of  safety. 

In  about  half  the  cases  there  is  no  aura  at  all. 

Indefinite  feehngs,  headaches,  depression,  and  irrita- 
bility sometimes  enable  the  patient  to  prophesy  the  onset 
of  a  fit  some  hours,  or  even  a  day  or  two,  before  its 
occurrence. 

2.  Motor  aurse. — These  consist  of  twitching  of  parts 
of  limbs,  and  where  the  extension  of  the  discharge 
proceeds  slowly  its  progress  can  be  traced  by  the 
movements  of  successive  parts  of  the  Hmbs,  just  as 
miy  be  done  when  a  tumour  causes  the  fit  (Jacksonian 
epilepsy).  It  is  important  to  emphasize  the  fact  that 
the  characteristics  of  Jacksonian  epilepsy,  where  the 
convulsion  spreads  slowly  from  part  to  part,  and  either 
remains  local,  or  becomes  general  with  loss  of  conscious- 
ness, are  by  no  means  confined  to  cases  where  there  is  a 
tumour  or  other  gross  lesion  (in  which  connection  the 


438    DISEASES   OF   THE  NERVOUS   SYSTEM 

term  was  first  used),  but  are  very  often  found  in  associa- 
tion with  attacks  of  "  idiopathic  "  epilepsy. 

Postepileptic  automatism. — Here  the  highest 
cerebral  functions  are  in  abeyance  and  the  patient  per- 
forms  coordinated  acts  without  any  knowledge  of  what 
he  is  doing  :  thus,  he  may  walk  for  hours,  and  on 
coming  to  his  senses  may  find  himself  miles  from 
where  he  intended  to  go  ;  or  he  may  drive  through 
crowded  cities  without  any  remembrance ;  or — most 
important  of  all — ^he  may  commit  any  kind  of  crime 
while  he  is  in  this  irresponsible  condition. 

The  important  point  in  this  connection  is  that  the 
automatism  is  just  as  likely,  or  perhaps  even  more 
lik(^ly,  to  follow  a  minor  fit  which  may  be  so  slight  as 
to  pass  unrecognized  by  those  around,  who  therefore 
only  see  in  the  illegal  act  the  impulse  of  the  criminal. 
Mental  disturbances  sometimes  appear  to  take  the  place 
of  a  fit,  and  are  then  spoken  of  as  "  psychic  equivalents." 

Postepileptic  mania  is  not  very  common.  It 
is  often  of  temporary  duration,  but  while  it  is  present 
the  patient  may  be  very  violent. 

Postepileptic  paralysis. — ^After  a  fit,  one  or  more 
limbs  are  sometimes  paralysed.  The  cause  is  presumably 
nervous  exhaustion,  and  the  power  generally  quickly 
returns.     It  is  not  a  common  occurrence. 

Details  of  a  fit. — It  will  now  be  instructive  to  follow 
out  a  fit  in  detail. 

If  the  patient  be  subject  to  a  visual  aura  the  discharge 
will  begin  in  the  occipital  area,  which  is  the  seat  of  the 
function  of  vision,  and  he  has  visual  sensations  which 
may  take  the  form  of  bright  lights  or  dimness  of  vision. 
The  face  will  probably  become  pale. 

The  discharge,  having  once  broken  down  the  sur- 
rounding resistance,  spreads  with  lightning  rapidity 
over  the  motor  and  frontal  regions.  The  part  of  the 
motor  area  which  generally  appears  to  feel  the  efiect 
first  is  that  connected  with  lateral  movements  of  the 
head  and  eyes,  and  hence  the  head  and  eyes  turn  to  one 
or  other  side  (conjugate  deviation).     Almost  synchron- 


EPILEPSY:    THE    FIT  439 

ously  with  this  movement  the  discharge  spreads  over  the 
rest  of  the  fore  part  of  the  brain.  Consciousness  is  lost, 
and  the  intense  stimulation  of  the  motor  area  throws 
the  whole  body  into  a  sustained  spasm.  The  patient 
becomes  quite  rigid  and  falls  like  a  log,  uttering  at  the 
same  time  a  cry,  groan,  or  gurgling  noise.  He  is  now 
in  the  stage  of  tonic  spasm,  in  which  the  muscles  of 
respiration  take  part,  and  consequently  the  face  becomes 
turgid  and  the  veins  swell.  Frothy  fluid  appears  at  the 
lips.  After  a  few  seconds  a  little  twitching  is  seen, 
beginning  first  probably  in  the  face  and  then  gradually 
becoming  general ;  the  limbs  are  alternately  violently 
flexed  and  extended ;  the  champing  jaw  often  catches  the 
protruding  tongue,  and  blood  is  added  to  the  frothy  mucus 
in  the  mouth.     The  sphincters  are  frequently  relaxed. 

Then  the  violence  of  the  movements  gradually 
diminishes,  until  in  two  or  three  minutes  or  so  all  is 
quiet  again,  and  the  patient  either  gets  up  and  goes 
about  his  business  in  a  more  or  less  dazed  condition, 
or,  if  the  circumstances  are  favourable,  sleeps  for  several 
hours. 

The  conjunctival  reflex  is  lost  throughout  the  fit, 
and  the  pupils  are  dilated  and  do  not  react  to  light. 

Immediately  after  a  severe  fit  the  knee-jerks  may 
be  temporarily  abolished  owing  to  the  exhaustion  of 
the  pyramidal  fibres  consequent  on  the  convulsion, 
but  more  often  the  knee-jerks  are  increased,  and  ankle- 
clonus  and  temporary  extensor  response  of  the  plantar 
reflex  may  be  present. 

Status  epOepticus. — ^In  the  status  epilepticus, 
which  is  a  rare  complication  of  epilepsy,  the  patient 
has  a  rapid  succession  of  fits,  and  in  the  more  severe 
forms  there  are  no  intervals  of  consciousness  between 
the  attacks.  It  is  always  a  serious  condition,  and  in 
many  cases  death  occurs  from  exhaustion. 

Minor  epilepsy. — Minor  attacks  are  variously 
designated,  either  as  "  faints,"  "  turns,"  or  as  "  dizzy  " 
and  "  peculiar  "  feehngs.  Apparently  they  are  due  to 
localized  discharges  of  momentary  duration,  and  their 


440    DISEASES   OF   THE   NERVOUS   SYSTEM 

signs  therefore  pass  off  rapidly  and  are  very  limited  in 
their  distribution.  The  most  common  variety  is  char- 
acterized by  a  momentary  loss  of  consciousness,  thus 
indicating  a  disturbance  of  the  highest  functional  level 
of  the  brain.  The  patient  "  loses  himself  "  for  a  few 
seconds,  stoj)S  abruj^tly  in  his  conversation  or  work, 
perhaps  drops  something,  and  then  continues  as  before. 
Such  attacks  often  pass  unnoticed,  but,  if  attention  is 
drawn  to  them,  a  j^assing  pallor  of  the  face,  with  possibly 
a  slight  twitching  of  some  muscles,  may  be  observed. 

The  "  reminiscences  "  to  which  allusion  has  already 
been  made  are  also  common  in  minor  epilepsy. 

Visceral,  auditory,  and  visual  sensations  are  all 
common,  and  indeed  any  of  the  sensations  which  have 
been  described  as  the  aura  of  the  major  fits  may  occur 
as  manifestations  of  the  minor  variety. 

In  this  way  the  minor  attacks  may  be  looked  upon 
as  abortive  fits  in  which  the  nervous  disturbance  remains 
strictly  circumscribed.  Presumably  these  slight  dis- 
turbances are  particularly  apt  to  arise  in  the  frontal 
region,  since  momentary  loss  of  consciousness  is  such  a 
common  event.  A  patient  may  suffer  from  minor 
attacks  for  years  before  a  major  fit  occurs  ;  in  other  cases 
both  frequently  occur,  but  in  many  the  major  attacks 
alone  occur. 

Postepileptic  automatism,  as  already  remarked, 
is  very  liable  to  fellow  a  minor  fit  which  may  have  been 
so  shght  as  to  pass  unnoticed. 

Diagnosis. — Epilepsy  has  to  be  distinguished  from 
convulsions  which  have  a  definite  organic  or  toxsemic 
basis. 

From  convulsions  of  infancy. — Little  children  are, 
as  we  have  already  seen,  very  hable  to  convulsions, 
which  in  the  majority  of  cases  cease  to  recur  as  develop- 
ment takes  place.  The  individual  convulsions  are  in- 
distinguishable from  those  of  epilepsy,  but,  bearing  in 
mind  this  special  tendency  of  childhood,  it  is  permissible 
to  hope  that  in  any  given  case  the  fits  will  pass  off  as  the 
child  grows  older.     Suspicions  may  be  aroused  by  the 


EPILEPSY:    DIAGNOSIS  441 

history  of  attacks  in  other  members  of  the  family,  and  by 
the  fits  occurring  in  an  apparently  quite  healthy  child 
without  any  adequate  associated  condition  such  as 
rickets. 

Lastly,  it  must  be  remembered  that  a  succession  of 
convulsions  in  childhood  undoubtedly  makes  for  the 
occurrence  of  fits  in  later  life,  and  it  is  common  to  find 
a  recurrence  of  the  convulsions  about  the  age  of  puberty, 
even  though  there  have  been  several  intervening  years 
of  freedom. 

From  convulsions  due  to  organic  disease  in  children. 
— If  the  organic  disease  is  acute,  e.g.  meningitis,  the 
convulsions  are,  of  course,  for  the  moment,  a  symptom 
of  minor  importance.  If  the  fits  are  associated  with 
any  of  the  forms  of  infantile  hemiplegia  or  diplegia, 
or  follow  as  a  late  result  of  meningitis,  or  from  hydro- 
cephalus, then  they  are  likely  to  assume  the  ordinary 
character  of  epilepsy. 

From  convulsions  due  to  cerebral  tumour. — There  are 
two  conditions  in  which  epilepsy  may  be  confused  with 
cerebral  tumour  : 

1.  When  the  convulsion  is  general. — Occasionally  it 
happens  that  a  general  convulsion,  indistinguishable 
from  an  epileptic  fit,  is  the  first  sign  of  a  cerebral  tumour. 
While,  of  course,  bearing  in  mind  the  possibility  of  other 
symptoms,  the  most  certain  way  to  avoid  mistaking 
the  generalized  convulsion  of  a  tumour  for  epilepsy  is 
by  examining  systematically  the  optic  discs.  It  is  very 
likely  that  the  appearance  of  the  fundus  will  be  sus- 
picious, even  if  there  are  no  other  symptoms  to  suggest 
the  true  nature  of  the  disease. 

2.  When  the  convulsion  is  local.— Tumours  situated  in 
the  motor  area  of  the  brain  cortex  cause  fits  (Jacksonian 
epilepsy)  which  begin  by  localized  movements  that 
dehberately  progress  to  other  parts  of  the  Hmb,  and  may 
or  may  not  terminate  in  a  general  convulsion  with  loss 
of  consciousness. 

It  must  be  remembered  that  many  cases  of  ordinary 
epilepsy  also  begin  in  this  way,  and  that  in  the  complete 


442    DISEASES   OF   THE   NERVOUS   SYSTEM 


absence  of  all  other  symptoms  of  a  tumour  the  case  is 
likely  to  be  one  of  idiopathic  epilepsy. 

From  convulsions  due  to  general  paralysis. — General 
paralytics  are  always  liable  to  convulsions,  and  the  possi- 
bility of  these  must  always  be  remembered  when  a  middle- 
aged  man  or  woman  suddenly  has  a  fit  for  the  first  time. 
In  some  instances  fits  appear  to  be  due  to  localized 
parenchymatous  syphilitic  changes  in  the  cerebral  cortex 
and,  as  such,  need  not  be  associated  with  any  clinical 
signs  to  indicate  the  more  widely  spread  changes  of 
general  paralysis. 

From  uraemic  convulsions. — Uraemia  sometimes  first 
shows  itself  by  a  convulsion,  but  an  examination  of  the 
urine  will  usually  make  the  nature  of  the  case  clear. 

From  convulsions  due  to  alcoholism. — Chronic  alcoholics 
sometimes  have  for  the  first  time  in  middle  life  a  fit  which 
appears  to  be  epilepsy,  though  in  many  it  is  probable 
that  the  effects  of  renal  disease  are  complicating  factors. 

From  convulsions  due  to  hysteria. — The  difficulty  of 
deciding  whether  a  fit  is  hysterical  is  chiefly  due  to  the 
fact  that  in  most  cases  the  medical  man  has  only  the 
history  to  depend  upon. 

The  chief  points  on  which  reliance  is  placed  in  making 
the  differentiation  are  as  follows  : — 


Epilepsy 

Hysteria. 

Cry     

A  single  cry  or  groan... 

Often  several  shrielcs. 

Fall 

(Sudden,    without    any 

Slide  down ;  seldom  hurt 

attempt  to  save  them- 

themselves by  falling. 

selves 

Sphincters     ... 

Often  relaxed  ... 

Not  relaxed. 

Tongue 

Bitten  ... 

Not  bitten,  hut  lips  may 

be. 
Often  struggle  and  fight, 

Struggling     . . . 

No     purposive     strug- 

gling    ' 

requiring  several  peo- 
ple to  hold  them 

Presence  of 

Fits  occur  when  patient 

Usually  in  presence  of 

others 

is   alone   or  in    pre- 
sence of  others 

others. 

Duration 

A.  few  minutes 

Often  a  long  time. 

EPILEPSY:    PROGNOSIS  443 

It  is  necessary  to  remember  that  an  hysterical  lit 
may  directly  succeed  one  of  epilepsy,  and  that  the 
latter  may  be  of  the  "  minor  "  kind,  and  be  quite  un- 
noticed by  the  ordinary  bystander. 

Prognosis. — ^It  is  difficult  to  estimate  with  any 
accuracy  the  number  of  cases  in  which  fits  have 
entirely  ceased,  for  such  patients  naturally  escape 
further  observation.  It  is  generally  thought  that  the 
disease  is  arrested  in  from  5  to  10  per  cent,  of  all 
cases.  It  is  impossible  to  predict  what  course  the 
disease  is  likely  to  take  until  the  effects  of  steady 
treatment  are  known,  for  it  is  rare  for  the  fits  to 
disappear  spontaneously. 

Long  intervals  between  fits,  and  absence  of  minor 
attacks,  are  both  favourable  points,  and  cases- beginning 
in  later  life  are  generally  more  easily  influenced  than 
those  arising  about  the  period  of  puberty.  Statistics 
show  that  the  prognosis  is  no  worse  in  cases  where  there 
is  an  hereditary  tendency.  The  principal  danger  to 
life,  except  when  the  rare  condition  of  status  epilepticus 
occurs,  arises  from  possible  accidents,  of  which  the  chief 
are  suffocation  from  turning  on  to  the  face  in  nocturnal 
epilepsy,  injuries  or  death  from  a  fall  when  conscious- 
ness is  lost,  and  choking  from  the  passage  of  vomited 
food  into  the  larynx.  In  a  considerable  number  of  con- 
firmed epile2:)tics  there  is  mental  deterioration,  which 
not  infrequently  becomes  advanced  enough  to  constitute 
insanity. 

Treatment. — To  treat  epilepsy  with  any  degree 
of  success  the  utmost  attention  must  be  given  to 
details. 

The  daily  fife  should  be  arranged  to  avoid  any  con- 
ditions which  are  likely  to  cause  sudden  variations  in  the 
equilibrium  of  the  nervous  system. 

Peripheral  stimuli  of  undue  strength  from  without, 
as  the  sudden  appearance  of  bright  lights,  loud  noises, 
and  other  things  causing  "  starts,"  must  be  avoided 
just  as  much  as  those  from  within,  and  a  quiet,  even 
life,  with  perfectly  regular  hours,  should  be  led.     The 


444    DISEASES   OF   THE   NERVOUS   SYSTEM 

occupation  should  be  physical  and  mental,  judiciously 
proportioned  to  each  case.  It  is  most  important  not 
to  allow  idling  and  day-dreaming.  Recognition  of  the 
possibility  of  psychological  factors  in  the  causation  of 
epilepsy  has  further  emphasized  the  importance  of 
broad  educational  treatment  as  a  means  of  improving 
the  general  mentality  of  patients  and  increasing  their 
powers  of  adaptation  to  the  general  stresses  of  life.  All 
evidence  shows  that  occupation  reasonable  in  kind  and 
degree  is  beneficial,  and  it  is  largely  due  to  the  opportun- 
ity of  leading  a  mechanical  life,  with  i)roper  occupation, 
that  epileptic  colonies  are  so  successful.  It  is  not 
common  for  epileptics  to  have  fits  when  they  are  engaged 
in  some  engrossing  occupation  ;  the  dangerous  moments 
seem  rather  to  be  those  in  which  the  mind  is  aimlessly 
wandering,  and  just  between  the  sleeping  and  the  waking 
states  is  a  favourite  time  for  a  fit. 

Arrest  of  fits  at  their  onset. — Many  patients  find  out 
for  themselves  that  they  can  frequently  ward  off  a 
threatening  fit  by  "force  of  will."  This  power  should 
be  encouraged,  and  it  may  often  be  aided  by  muscular 
exertion.  The  moment  a  fit  threatens,  the  patient 
should  clench  his  teeth  and  grasp  something  tightly. 
The  most  convenient  method  is  to  clasp  his  own  arm  as 
tightly  as  possible,  and  in  many  instances  the  discharge 
can  in  this  way  be  arrested. 

The  possibility  of  arresting  fits  by  tightening  a  cord 
when  the  aura  passes  up  a  limb  has  already  been  men- 
tioned (p.  436),  and,  when  practicable,  should  always 
in  such  cases  be  employed.  Smelling  strong  salts  and 
inhalations  of  amyl  nitrite  sometimes  meet  with  success. 
Every  fit  stopped  is  a  distinct  gain,  and  where  the 
fits  are  few  and  far  between  it  is  most  important  to 
make  the  gaps  as  wide  as  possible. 

Diet. — The  food  should  be  simple  and  quite  plainly 
cooked,  and  spices  of  all  kinds  should  be  avoided. 
The  question  of  allowing  meat  is  one  on  which 
there  is  some  difference  of  opinion.  Certainly,  the 
meat   taken    should    be  moderate    in   amount   and    of 


EPILEPSY:    TREATMENT  445 

an  easily  digested  nature,  but  there  seems  to  be  no 
great  benefit  in  abolishing  it  altogether,  at  least  in 
the  majority  of  cases.  More  recently  it  has  been 
thought  that  the  harmful  constituents  of  food  are 
the  "  purin  "  bodies,*  and  diets  have  been  constructed 
to  ehminate  these.  Such  a  diet  would  include  milk, 
eggs,  butter,  cheese,  rice,  macaroni,  tapioca,  white 
bread,  cabbage,  lettuce,  sugar,  and  fruit,  while  in 
potatoes  and  onions  the  amount  of  purin  bodies  is  very 
small.  Tea,  cocoa,  coffee,  meat,  and  fish  should,  on 
this  hypothesis,  be  avoided.  Among  the  meats  which 
contain  the  least  quantities  of  purin  bodies  are  tripe, 
neck  of  pork,  and  cod-fish. 

Aldren  Turner  f  has  found  that  diets  constructed 
on  this  principle  seem  to  be  beneficial  in  some  instances, 
but,  like  other  forms  of  treatment,  are  not  likely  to 
produce  an  effect  that  is  constant  for  all  cases. 

Elimination  of  salt. — The  elimination  of  salt  from 
the  diet  has  been  advised  on  the  supposition  that  the 
bromide  can  take  its  place  in  the  tissues  of  the  brain. 
By  this  means  it  was  hoped  that  the  fits  might  be  con- 
trolled by  smaller  doses  of  bromides,  which  at  the  same 
time  would  have  a  more  powerful  effect.  No  constant 
results  have  been  obtained,  but  a  certain  proportion  of 
cases  seem  to  have  benefited,  so  that  it  is  often  worth 
a  trial. 

Drink. — Alcohol  in  any  form  should  be  absolutely  for- 
bidden. Coffee  is  too  stimulating;  but  weak  fresh  tea 
may  generally  be  taken.  Water  and  milk  are  the  best 
drinks  for  general  purposes. 

Bowels. — The  bowels  should  be  most  carefully  regu- 
lated. Constipation  very  often  appears  to  be  the  exciting 
cause  of  an  attack. 

Drugs. — The   bromides   of    potassium,    sodium,    and 

*  The  puriu  bodies  are  substances  of  which  the  composition  C5'N'4 
forms  the  basis ;  they  are  fouud  in  many  common  foods,  and  also  as 
the  result  of  tissue  metabolism  of  the  body.     See  "Purin  Bodies  of 
Food-Stuflfs,"  by  Walker  Hall, 
t  Fractitioner,  April,  1906. 


446    DISEASES   OF   THE   NERA^OUS   SYSTEM 

ammonia,  given  judiciously,  are  the  most  useful  drugs  in 
epilepsy,  and,  in  spite  of  its  more  depressing  influence, 
potassium  bromide  is  the  one  which  appears  on  the  whole 
to  give  most  satisfaction.  Many  prefer  to  give  the 
different  bromides  together.  Bromide  of  strontium  a|so 
is  useful. 

Regulation  of  dose  of  the  bromides. — Much  of  the 
success  in  treatment  depends  upon  carefully  regulating 
the  doses.  If  too  little  is  given,  the  fits  are  not  checked  ; 
while  if  the  amount  is  unnecessarily  large,  mental 
depression  and  dullness  are  produced.  The  ideal  dose 
is  that  which  is  just  sufficient  to  check  the  fits,  but  it  is, 
of  course,  impossible  to  lay  down  any  rules,  as  so  much 
depends  upon  the  effects — good  or  bad — of  bromides  in 
every  individual  case.  Broadly  speaking,  the  dose 
limits  of  bromides  in  epilepsy  are  between  10  and  80 
grains  a  day.  Larger  doses  are  sometimes  needed,  but 
as  a  rule  are  not  beneficial  when  the  smaller  amount  has 
no  good  effect. 

Time  for  giving  bromides. — Much  may  be  done  by 
considering  the  times  at  which  fits  usually  occur  in  rela- 
tion to  the  taking  of  medicines.  Where  the  attacks 
show  no  regularity  the  doses  may  be  taken  at  con- 
venient intervals  twice  or  three  times  a  day  as  required, 
but  where  it  is  possible  to  calculate  to  some  extent 
on  the  onset  of  a  fit,  a  judicious  anticipation  will 
often  meet  with  success.  Thus,  if  the  fits  occur  only 
during  sleep,  or  in  early  morning,  a  single  dose  of 
20  or  30  grains  of  bromide  may  be  taken  the  last 
thing  at  night. 

Drawbacks  of  bromides. — The  drawbacks  to  taking 
bromides  continuously  are  the  tendency  to  the  production 
of  (1)  acne,  (2)  mental  depression. 

The  tendency  to  acne  can  be  counteracted  by  com- 
bining small  doses  of  arsenic  with  the  bromide.  This 
in  its  turn  has  a  drawback,  for  arsenic  taken  over  a  long 
period  of  time  may  give  rise  to  pigmentation  of  the  skin. 
When  combined  with  bromide  the  arsenic  in  the  doses 
usually  given  has  not  the  same  tendency  to  produce 


EPILEPSY:    TREATMENT  447 

neuritis  or  other  unpleasant  effects  which  it  is  sometimes 
apt  to  do  when  taken  in  other  ways. 

An  example  of  a  prescription  is  as  follows  : — 


Potassii  bromidi    ...       Dg 

12       . 

••     Sr- X 

Liquoris  arseniealis      '  CI 

12 

. .     in.ii 

Spiiitus  chlorofonni       Ml 

1 

..    in  XV 

Aquam         ...          ...  ad  Ml 

30       . 

■  •     si 

Two  tablespoonfuls  to  be  taken  twice  or  tbree  times  a  day 
after  food. 

As  already  mentioned,  the  bromides  of  sodium  or 
ammonia  may  be  substituted  for  that  of  potassium, 
or  the  dose  may  be  divided  between  the  three. 

Adjuvants  to  hromides. — While  bromides  form  the 
basis  of  nearly  every  prescription  for  epilepsy,  there  are 
other  drugs  which,  when  combined  with  them,  appear 
often  to  have  some  good  influence.  Of  these  the  most 
important  are  belladonna  and  borax,  either  or  both 
of  which  may  be  combined  with  the  bromides 

V^   Potassii  bromidi    ... 
Boracis  purificati  ... 
Tincturse  belladonnas 
Liquoris  arseniealis 
Spiritus  chloroformi 
Aquam         ...         ...  a 

Two  tablespoonfuls  to  be  taken  three  times  a  day. 

Occasionally  the  addition  of  small  doses  of  digitalis 
and  potassium  iodide  seems  to  have  a  good  effect.  Iron 
may  be  of  assistance  when  there  is  anaemia,  but  in  some 
cases  it  disagrees.  Nitro- glycerin  may,  in  the  form  of 
liquor  trinitrini,  be  given  with  the  bromides  when  the 
condition  of  the  peripheral  circulation  seems  to  warrant 
the  administration  of  vaso-dilators.  Sodium  luminal  (a 
derivative  of  veronal)  has  recently  been  used  with  good 
results  in  some  cases.  A  usual  dose  is  1  gr.  for  an 
adult  and  \  gr.  for  a  child,  twice  a  day. 

The  minor  fits  are  often  more  difficult  to  control 
than  the  major  ones;  and  where  bromides  fail,  pills 
of  zinc  oxide  may  be  tried.  An  endless  number  of  drugs 
has  naturally  been  tried   and  commended  at   various 


Dg 

12 

..     gr.  XX 

Dg 

3 

..      gr.  V 

Dl 

3 

..     inv 

CI 

12 

, ..     ntii 

Ml 

1 

..       ll^XV 

Ml 

30 

...     §i 

448    DISEASES   OF   THE   NERVOUS   SYSTEM 

times,  but  up  to  the  present  no  mixture  has  met  with 
such  success  as  those  of  which  bromides  constitute  the 
basis. 

Surgical  treatment,  often  useful  where  fits  depend 
upon  a  gross  lesion,  is  very  seldom  indeed  of  use  in  general 
epilepsy.  Here  and  there  a  very  local  and  deliberate 
onset  may  suggest  the  advisability  of  removing  a  piece 
of  the  cortex,  but  in  the  large  majority  of  cases  no 
operation  is  likely  to  produce  a  cure. 

Summary  of  treatment. — The  points  to  which  atten- 
tion should  chiefly  be  given  may  be  summed  up  as 
follows  : — 

1.  General  mode  of  living. 

2.  Diet. 

3.  Alimentary  canal. 

i.  Removal  of  all  sources  of  peripheral  irritation. 

5.  Administration  of  drugs. 

6.  Education  and,  in  suitable  cases,  psychothera- 
peutic treatment. 


CHAPTER   XLVI 

TETANY 

Tetany  is  characterized  by  spasmodic  tonic  contractions 
of  the  muscles,  chiefly  those  of  the  extremities,  and  by 
an  increase  in  the  irritability  of  the  muscles  to  electrical 
and  mechanical  stimulation. 

Etiology. — Tetany  is  most  commonly  to  be  seen  in 
children  who  are  the  subjects  of  rickets.  More  rarely 
it  occurs  in  adults,  and  has  been  noted  in  association 
with  chronic  dilatation  of  the  stomach,  diarrhoea, 
debility  after  lactation,  and  removal  of  the  thyroid 
gland,  in  connection  mth  which  the  parathyroid  is  sup- 
posed to  be  the  essential  factor.  Occasional  epidemics 
have  been  recorded. 

Pathology. — The  contractions  are  probably  set  up 
by  a  toxaemia  which  arises  at  some  time  during  the 
course  of  the  diseases  mentioned  above.  No  definite 
lesions  have  been  found  after  death,  and  the  exact  seal 
of  the  irritative  process,  whether  in  brain,  cord,  or  nerves.. 
is  a  matter  which  still  remains  undecided. 

Symptoms. — The  spasms,  which  are  often  preceded 
by  sensations  of  tingling  and  numbness,  are  generally 
most  prominent  in  the  distal  parts  of  the  extremities. 
The  hand  usually  assumes  an  attitude  which  is  character- 
istic. The  fingers  are  flexed  at  the  metacarpo-phalangeal 
joints  and  extended  beyond,  a  position  which  is  brought 
about  by  a  spasm  of  the  interossei,  while  the  thumb 
is  brought  into  the  position  of  opposition  and  pressed 
tightly  into  the  palm.  At  other  times  the  fingers  assume 
the  shape  of  the  "claw-hand."  The  wrist-  and  elbow- 
2d  449 


450    DISEASES   OF   THE   NERVOUS   SYSTEM 

joints  are  flexed,  and  the  arm  is  adducted.  Tonic 
spasms  of  a  corresponding  nature  occur  m  the  feet ; 
the  foot  is  inverted  and  the  toes  are  flexed.  Swelling 
of  the  hands  and  feet  sometimes  occurs,  and  the  attacks 
may  also  be  accompanied  by  a  considerable  amount  of 
pain  and  tenderness  in  the  limbs.  In  severe  cases  the 
spasms  spread  to  the  trunk  and  cause  opisthotonos,  and 
in  some  instances  the  tongue  and  muscles  of  the  face 
are  affected. 

The  attacks  last  for  variable  times.  Sometimes  the 
spasms  pass  off  in  a  few  minutes,  and  at  others  they 
are  more  or  less  continuous  for  some  hours. 

Trousseau  observed  that  an  attack  may  be  brought 
on  by  pressure  on  the  main  nerves  and  vessels  of  the 
limb. 

The  muscles  and  their  motor  nerves  are  very  irrit- 
able to  mechanical  stimulation  (Chvostek's  sign),  a  fact 
which  can  be  well  observed  in  connection  with  the 
muscles  of  the  face.  The  sensory  nerves  also  are  unduly 
irritable. 

The  excitability  of  the  nerves  to  electrical  stimula- 
tion, especially  to  that  of  the  galvanic  current,  is  greater 
than  normal,  and  the  anodal  opening  contraction  is 
greater  than  the  kathodal  closure  contraction. 

Diagnosis.  —  The  carpo  -  pedal  contractions  of  a 
typical  case  are  not  likely  to  be  confused  with  any- 
thing else. 

When  the  spasms  extend  to  the  trunk  and  face,  the 
possibility  of  tetanus  may  present  itself,  but  is  negatived 
by  the  order  in  which  the  contractions  have  appeared, 
as  well  as  by  the  absence  of  trismus,  which  is  such  a 
characteristic  early  sign  of  tetanus. 

Prognosis. — The  occurrence  of  tetany  in  children 
is  not  usually  dangerous,  but  sudden  death  has  occasion- 
ally taken  place  in  association  with  the  spasms.  As  a 
complication  of  chronic  dilatation  of  the  stomach, 
tetany  may  be  very  troublesome  or  even  dangerous  ; 
and  if  organic  disease  of  the  stomach  be  present,  the 
onset    of    the    spasms    may    indicate    the    immediate 


TETANY  451 

necessity  of  obtaining  some  relief  througli   a   surgical 
operation. 

Treatment. — In  children  the  bowels  should  be 
relieved  by  castor  oil,  and  enemata  if  necessary.  Warm 
baths  may  be  useful  during  an  attack.  Among  the 
sedatives  that  are  most  likely  to  bring  relief  are  bromides 
and  chloral.  Attention  must  also  be  paid  to  the  general 
health,  which  in  the  case  of  children  is  usually  impaired 
by  rickets. 


CHAPTER  XLVII 

PARALYSIS    AGITANS    (PARKINSON'S   DISEASE) 

Paralysis  agitans  is  characterized  by  the  gradual 
onset  of  weakness,  tremors,  and  rigidity. 

Some  degree  of  weakness  is  probably  present  in  all 
cases,  but  the  amount  of  tremor  and  rigidity  is  very 
variable. 

From  the  point  of  view  of  diagnosis,  rigidity  is  quite  as 
important  a  symptom  as  tremor,  and  the  nature  of  the 
disease  is  often  clear  though  there  may  be  no  tremor. 

Etiology. — ^It  is  not  possible  to  attribute  the  onset 
to  any  direct  cause,  though  injury,  shock,  worry,  or 
anything  that  causes  general  debility  sometimes  appears 
to  be  the  starting-point.  Between  45  and  60  is  the 
age  at  which  the  disease  usually  begins,  and  men  are 
rather  more  commonly  affected  than  women.  Symp- 
toms indistinguishable  from  those  of  paralysis  agitans 
have  recently  been  observed  as  occasional  results  of 
encephalitis  lethargica  {see  p.  344). 

Pathology. — Nothing  definite  is  known  concerning 
the  underlying  changes  in  the  nervous  system.  Altera- 
tions in  the  structure  of  the  cortical  cell  bodies  and  their 
dendrons  have  been  found  by  some,  while  others  have 
described  arterial  and  perivascular  changes  ;  but  none 
of  these  seems  to  be  constantly  present.  It  has  been 
suggested  that  the  modification  or  want  of  some  internal 
secretion,  more  especially  of  that  connected  with  the 
parathyroids,  may  be  the  cause.  Others  consider  that 
the  essential  changes  take  place  in  the  muscles.  Wilson 
has  remarked  upon  the  resemblance  that  exists  between 
the  symptoms  of  paralysis  agitans  and  those  of  progressive 

452 


PAKALYSIS   AGITANS  453 

lenticular  degeneration  {see  p.  386).  Tremors,  rigidity, 
and  weakness  are  common  to  both  conditions,  and  in 
these  circumstances  Wilson  considers  it  might  be  expected 
that  the  localization  of  the  lesions  should  be  more  or 
less  identical,  i.e.  in  the  corpus  striatum.  Jelgersma  is 
quoted  as  having  demonstrated  degeneration  of  strio- 
thalamic  fibres  and  of  the  lenticular  bundle  of  Forel  in 
paralysis  agitans. 

In  any  case,  the  frequency  with  which  the  onset  of 
symptoms  assumes  a  hemiplegic  distribution  is  strongly 
suggestive  of  the  cerebral  origin  of  the  disease. 

Symptoms. — The  onset  is  usually  insidious.  Oc- 
casionally the  tremor  seems  to  date  definitely  from  the 
time  of  an  injury  or  shock,  but  more  often  it  begins  so 
gradually  as  to  be  scarcely  noticeable. 

In  some  a  slow,  progressive  weakness,  with  increasing 
stiffness,  marks  the  onset ;  while  in  others  the  weakness, 
tremors,  and  rigidity  all  appear  at  about  the  same  time. 


Pig.  109.— Writirig  in  a  case  of  paralysis  agitans. 

The  tremors  are  generally  first  noticed  in  one  hand  ; 
the  thumb  and  forefinger  move  rhythmically  in  a  way 
which  has  been  aptly  described  as  "  pill-rolling "  in 
character.  At  first  the  trembling  is  scarcely  noticeable 
but  gradually  obtrudes  itself  until  it  begins  to  interfere 
with  ordinary  actions  such  as  writing.     (Fig.  109.) 

From  the  forefinger  and  thumb  the  tremor  spreads 
to  the  other  fingers,  and  at  last  the  whole  hand  takes 
part  in  the  movement,  which  later  may  extend  to  the 
elbow  and  to  some  extent  to  the  shoulder. 

By  an  effort  of  will  the  movements  can  be  controlled, 
but  only  for  a  few  seconds,  and  in  severe  cases  an  attempt 
to  keep  one  limb  still  generally  results  in  increased  move- 
ments of  another. 

The  tremors  are  most  obvious  when  the  limbs  are  at 


454    DISEASES  OP  THE  NERVOUS  SYSTEM 

rest,  and,  indeed,  during  brief  voluntary  movements,  e.g. 
holding  out  the  hand,  they  become  distinctly  less,  though 
they  do  not  altogether  cease,  as  is  sometimes  stated. 

This  remission  during  voluntary  movement  can  be 
easily  demonstrated  by  asking  the  patient  to  write 
or  draw  a  line,  an  action  which,  from  the  tremor  present, 
would  often  appear  almost  impossible,  but  which  on 
trial  often  proves  to  be  done  comparatively  well  (Fig.  110). 

The  tremors  cease  during  sleep. 

From  one  hand  the  tremor  generally  spreads  to  the 
leg  on  the  same  side,  and  thence  to  the  hand  on  the 
other  side,  but  there  are  many  exceptions  to  this  rule. 
Some  of  these  are  more  apparent  than  real,  for  the 
tremor  in  the  legs  is  often  less  noticeable  than  that  in 
the  arms,  probably  because  the  muscles  are   more  in 


Fig.  110.— Line  drawn  by  a  patient  witla  a  severe  tremor,  showing 
at  the  beginning  power  temporarily  to  control  the  tremor. 

action — movement,  as  already  mentioned,  becoming  more 
obvious  when  the  muscles  are  relaxed. 

As  the  disease  progresses,  the  head,  face,  and  jaws 
may  all  take  part  in  the  movements. 

Rigidity  is  even  more  characteristic  of  paralysis 
agitans  than  tremor. 

At  first  scarcely  noticeable,  it  creeps  on  relentlessly 
until  the  figure  becomes  firmly  set  in  the  characteristic 
position  (Fig.  111).  Obeying  the  usual  laws,  the  flexors 
overbalance  the  extensors,  and  the  patient  becomes 
bowed  like  an  old  man,  with  the  head  bent  forwards 
and  the  body  flexed  The  arms  are  adductcd  at  the 
shoulder  and  flexed  at  the  elbows,  while  the  fingers 
and  thumb  are  bent  into  a  half-closed  position.  The 
thumb  is  often  curved  in  a  position  of  hvperextension 
(Fig.  112). 

The  features  are  fixed  and  expressionless  {Parkinson's 
mask).  The  voice  is  dull  and  monotonous,  owing 
chiefly  to  the  rigidity  of  the  muscles  of  the  lips  and  face. 


PARALYSIS   AGITANS 


455 


It   cm    easily  be  imitated  by  trying  to  talk  without 
moving  the  lips. 


Pig.  Ill —Case  of  paralysis  agitans  showing  the  characteristic 

attitude. 

Voluntary   movements   are   performed    slowly   and 
stiffly,  and  the  reaction  times  are  thereby  lengthened. 


456    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  relaxation  of  muscles  also  takes  place  more  tardily 
than  usual. 

The  rigidity  of  the  bowed  body  with  the  upper  part 
bent  over  throws  the  centre  of  gravity  forward  and  leads 
to  the   peculiar  gait,  in  which  the  patient  takes  short, 


Fig.  112.— HyperexteDsion  of  the  thumb  in  a  case 
of  paralysis  agitaus. 

rapid  steps  that  oftentimes  amount  to  a  run,  and  when 
once  started  he  has  considerable  difficulty  in  stopping. 
From  his  appearance  it  has  been  well  said  that  the 
patient  looks  as  if  he  were  trying  to  catch  up  his  centre 
of  gravity. 


PARALYSIS    AGITANS  457 

The  mechanism  of  this  peculiar  gait  can  be  best  under- 
stood by  imitating  the  conditions  under  which  it  occurs. 
If  the  body  is  bent  well  over  and  kept  stiff,  so  that  the 
balance  is  only  just  preserved,  and  a  short  step  forwards 
is  taken,  there  will  be  a  tendency  to  fall,  which  is  only 
counteracted  at  each  step  by  rapidly  shufEing  the  hind 
foot  forwards.  This  movement  is  repeated  indefinitely, 
and  the  patient  being  unable  to  bend  the  body  back,  and 
so  to  restore  the  balance,  there  is  difficulty  in  stopping 
without  running  the  risk  of  falling,  unless  he  catches 
hold  of  something  for  support. 

In  the  same  way,  if  the  patient  is  suddenly  started 
backwards,  the  rigidity  of  his  body  makes  it  difficult  to 
regain  the  balance,  and  the  movement  backwards  is 
involuntarily  continued  for  some  steps.  This  is  known 
as  retropuhion.  A  similar  effect  can  also  be  obtained 
by  suddenly  starting  the  patient  on  a  side  movement 
[lateropulsion). 

The  deep  reflexes  are  generally  said  to  be  unaltered, 
but  in  many  cases,  especially  before  rigidity  becomes 
marked,  some  exaggeration  may  be  found.  Likewise, 
with  regard  to  the  plantar  reflexes  there  is  usually 
no  alteration,  but  systematic  examination  has  shown 
that  every  now  and  then  an  instance  occurs  in  which 
a  typical  extensor  response  is  obtained.  Taken  in  con- 
junction with  the  view  already  mentioned,  that  the 
seat  of  the  disorder  is  in  the  corpus  striatum,  this 
behaviour  of  the  reflexes  is  interesting,  since  it  shows 
that  the  degeneration,  although  primarily  outside  the 
pyramidal  motor  tracts,  may  occasionally  extend  into 
them,  and  so  cause  exaggerated  knee-jerks  and  extensor 
responses  to  be  added  to  the  usual  symptoms  of  the 
disease. 

Among  other  symptoms  are  cramps,  which  are  often 
troublesome.  Some  patients  complain  of  the  toes  draw- 
ing up  under  the  feet  and  troubling  them  in  walking. 

Sensations  of  heat  and  cold  are  common,  and  are 
apparently  of  vaso-motor  origin. 

In  the  later  stages,  when  it  is  almost  impossible  to 


458    DISEASES   OF   THE  NERVOUS  SYSTEM 

move  voluntarily,  there  is  often  very  great  restlessness, 
with  a  desire  to  have  the  position  of  the  limbs  constantly 
altered. 

Prognosis. — The  disease  progresses  slowly,  and 
finally  the  patient  may  be  quite  bedridden.  There  is  no 
special  danger  to  life. 

Treatment. — The  only  chance  of  doing  anything 
for  these  cases  is  to  begin  treatment  early.  Even  then 
it  is  only  palliative,  but  undoubtedly  much  comfort  and 
some  retardation  of  symptoms  can  often  be  obtained 
by  judicious  treatment. 

This  has  to  be  directed  towards  relief  of  the  three 
main  symptoms,  tremors,  rigidity,  and  weakness. 

The  cases  in  which  the  tremors  are  severe  are  the 
most  painful,  for  the  patient  is  never  able  to  be  at  rest, 
and,  unfortunately,  no  drugs  can  be  depended  upon  to 
still  them.  All  the  usual  sedatives  may  be  tried,  but 
only  seldom  is  any  lasting  effect  produced.  Opium 
and  its  preparations  are  of  no  use. 

When  the  tremors  are  very  bad,  hyoscine  sometimes 
checks  them  to  some  extent.  It  may  be  given  in  doses 
beginning  with  -^l^j  gr.  of  the  hydrobromide,  but  it  must 
be  used  with  caution  on  account  of  its  toxic  effects ;  and 
it  cannot  be  said  that  any  great  relief  is  often  obtained 
from  this  or  any  other  drug. 

In  slight  cases  the  patient  should  be  encouraged  to 
control  the  tremor,  but  in  the  more  severe  any  sustained 
effort  is  very  exhausting.  Little  devices  of  various 
kinds  can  sometimes  be  found  to  give  comfort.  In  one 
patient,  carrying  a  heavy  stick  gave  some  relief  to  the 
tremor. 

It  is  scarcely  necessary  to  state  that  as  quiet  a  life 
as  possible  should  be  led.  Excitement  makes  the 
tremor  worse,  and  in  some  instances,  so  long  as  the 
patient  remains  quiet,  it  gives  him  very  little  trouble 
for  quite  a  number  of  years. 

The  rigidity  is  best  combated  by  carefully  regulated 
active  and  passive  movements,  and,  if  these  are  prac- 
tised early  enough,  there  is  no  doubt  that  the  tendency 


PAEALYSIS   AGITANS  459 

to  permanent  fixation  may  be  postponed.  Hot  baths 
regularly  given  and  accompanied  by  light  massage  also 
afford  relief. 

The  weakness  requires  no  special  form  of  treatment. 
The  main  point  is  to  keep  up  the  general  nutrition  : 
the  diet  should  be  suitable  in  quantity  and  quality  ; 
and  the  patient  should  live  as  much  as  possible  in  the 
open  air — a  mode  of  life  which  seems  to  exercise  a  general 
influence  in  retarding  the  progress  of  the  disease. 


CHAPTER  XLVIIl 

CHOREA   (RHEUMATIC    CHOREA,    SYDENHAM'S 
CHOREA,   ST.  VITUS 'S   DANCE) 

Chorea  is  characterized  mainly  by  the  presence  of 
involuntary  movements,  together  with  varying  degrees 
of  muscular  weakness  and  incoordination.  Frequently 
there  are  also  some  mental  changes  present. 

Etiology. — Chorea  is  a  disease  chiefly  of  children 
between  the  ages  of  5  and  15,  and  occurs  more  often 
in  girls  than  in  boys. 

Rheumatism  is  undoubtedly  the  principal  under- 
lying cause,  and  statistics  show  that  a  definite  history 
of  rheumatic  manifestations  can  be  obtained  in  a  very 
large  number  of  cases.  Less  often  an  attack  of  rheu- 
matism follows  chorea,  and  only  occasionally  are  the 
two  conditions  associated. 

Acute  specific  fevers  other  than  rheumatism  are 
not  directly  concerned  in  the  causation  of  chorea.  The 
apparent  relationship  of  the  disease  to  scarlet  fever  may 
depend  upon  the  close  association  which  exists  between 
the  latter  affection  and  rheumatism. 

In  the  rheumatic  child  anything  which  strains  the 
nervous  system  seems  to  dispose  to  an  attack  of  chorea  ; 
hence  inherited  nervousness,  too  much  schooling,  frights, 
errors  of  refraction,  and  other  forms  of  irritation  often 
appear  in  the  history  of  the  case  as  the  apparent  causes. 
Fright  is  especially  apt  to  be  closely  followed  by  chorea. 

Chorea  is  sometimes  associated  with  pregnanci/,  but 
in  the  majority  of  these  cases  also  some  history  of 
previous  rheumatism  can  be  obtained. 

4G0 


CHOREA  461 

Pathology. — That  this  foim  of  chorea  is  a  mani- 
festation of  rheumatism  is  now  generally  conceded. 
Poynton  and  Holmes*  consider  that  the  Diflococcus 
rheumaticus,  the  presence  of  which  they  have  demon- 
strated in  the  pia  mater  of  fatal  cases,  is  probably  the 
infective  agent,  and  among  the  microscopic  changes  that 
have  been  observed  are  hypersemia,  thrombosis,  swelling 
and  chromatolysis  of  the  cortical  cells. 

Clinical  signs  which  indicate  the  presence  of  organic 
changes  in  the  nervous  system  have  also  been  frequently 
observed,  so  that  the  disease  cannot  any  longer  be 
classified  among  those  of  "  functional "  origin,  but,  as 
suggested  by  Grenet  and  Loubet,t  must  rather  be  re- 
garded as  a  form  of  infective  encephalitis. 

Symptoms. — The  onset  is  often  insidious,  and 
commences  wdth  "  inattention,"  "  dropping  things,"  and 
"  fidgeting."  Sometimes,  especially  after  a  severe  emo- 
tional disturbance,  the  onset  is  acute,  and  either  weak- 
ness or  irregular  movements  appear  quite  suddenly. 

The  symptoms  may  be  divided  into  the  following 
groups  : — 

1.  Involuntary  movements. 

2.  Muscular  weakness. 

3.  Incoordination. 

4.  Mental  changes. 

5.  Associated  rheumatic  manifestations. 

1.  The  movements. — The  movements  are  spasmodic, 
involuntary,  and  irregular.  They  may  be  distributed 
all  over  the  body,  or  they  may  be  confined  to  one 
limb  or  to  one  side  {Jiemichorea) .  Their  intensity  varies 
in  degree  :  in  one  case  it  may  be  quite  impossible  to 
keep  the  patient  in  an  ordinary  bed  ;  in  another  the 
movements  can  only  be  detected  with  difficulty.  In 
slight  cases  the  movements  generally  become  more 
obvious  if  the  child  holds  out  both  hands  with  the 
fingers  widely  outspread,  and  at  the  same  time  pro- 
trudes the  tongue. 

*  Lancet,  Oct.  13,  1906         f  Revue  Nevrologique,  Dec.  15^,  1912. 


462    DISEASES   OF   THE   NERVOUS   SYSTEM 

Irregularity  is  a  characteristic  of  the  movements, 
and  the  same  movement  is  never  repeated  continually, 
as  it  is  in  the  tics.  The  hands  are  generally  more  severely 
affected  than  the  upper  arm,  and  when  the  fingers  are 
spread  out  there  is  a  tendency  to  hyperextension.  Peculiar 
grimaces  are  made,  which,  together  with  spasms  of  the 
tongue  and  jaw,  often  make  talking  and  eating  difficult. 

The  diaphragm  may  contract  spasmodically,  and 
the  heart  may  beat  irregularly.  Excitement  of  any 
kind  makes  the  movements  worse,  and  in  severe  cases 
the  whole  body  is  tossed  about  with  great  violence. 
During  sleep  the  movements  disappear. 

2.  Loss  of  power. — There  is  probably  some  weak- 
ness in  almost  every  case  of  rheumatic  chorea,  but  it  is 
often  difficult  to  differentiate  it  from  the  spasmodic 
movements  and  incoordination,  both  of  which  cause 
apparent  loss  of  power  in  the  holding  of  objects. 

Loss  of  power  in  one  or  more  limbs  is,  however,  an 
important  feature  in  a  considerable  percentage  of  cases, 
and  occasionally  it  is  the  predominating  one,  in  which 
case  the  term  paretic  chorea  is  sometimes  used.  At  first 
sight  such  cases  may  simulate  the  paresis  of  acute  anterior 
poliomyelitis. 

3.  Incoordination. — There  is  considerable  difficulty  in 
carrying  out  voluntary  movements  with  precision,  as 
is  easily  seen  by  asking  the  child  to  perform  some 
delicate  act,  such  as  picking  up  a  tiny  object,  when  the 
fingers  move  irregularly  round  it  before  it  is  finally 
seized. 

4.  Mental  changes. — Dullness,  want  of  attention,  and 
inability  to  do  lessons  are  often  noticed.  In  bad  cases, 
especially  when  it  is  difficult  to  feed  the  child,  sleepless- 
ness may  be  a  troublesome  symptom.  In  the  severe 
cases  there  may  be  acute  delirium  accompanied  by  a 
high  temperature. 

There  are  no  definite  sensory  changes  peculiar  to 
chorea.  Occasionally  there  is  some  degree  of  anaesthesia 
of  varied  distribution,  and  the  disease  may  sometimes 
be  further  complicated  by  the  presence  of  hysterical 


CHOREA:    SYMPTOMS  463 

manifestations.     In  some  cases,  too,  complaint  is  made 
of  pains  in  the  limbs. 

5.  Associated  rheumatic  manifestations. — Endocarditis  is 
the  most  important  rheumatic  manifestation  that  fre- 
quently accompanies  chorea  ;  the  mitral  valve  is  the 
one  that  is  usually  affected,  and  a  systolic  murmur  can 
generally  be  heard  at  the  apex.  In  fatal  cases,  bead- 
like  vegetations  of  recent  origin  may  be  found  upon 
the  cusps. 

Erythema  nodosum  may  be  present  in  conjunction 
with  the  chorea. 

Joint  troubles  are  more  likely  to  have  occurred  at 
some  previous  time,  but  occasionally  they  are  met  with 
during  the  attack. 

Careful  examination  frequently  reveals  the  presence 
of  signs  that  are  usually  associated  with  organic  changes 
in  the  central  nervous  system.*  Some,  e.g.  an  extensor 
response  and  hypotonus  of  the  muscles,  are  indicative 
of  lesions  of  the  pyramidal  tracts,  while  others,  as  the 
inability  to  perform  successive  movements  such  as 
pronation  and  supination  of  the  forearm  rapidly 
(adiadocJiohinesis) ,  and  failure  to  arrest  the  movement  of 
the  finger  at  a  given  spot  (asynergia),  suggest  a  deficiency 
of  the  functions  of  the  cerebellum. 

The  irritative  nature  of  the  infection  is  further  shown 
by  lymphocytosis,  which  has  sometimes  been  observed 
in  the  cerebro-spinal  fluid  after  lumbar  puncture. 

The  knee-jerks  may  be  di£&cult  to  obtain,  but  when 
elicited  they  are  often  rather  exaggerated.  Sometimes, 
owing  to  the  irregular  contraction  of  the  quadriceps 
extensor,  the  leg  hangs  in  the  air  longer  than  usual,  a 
condition  which  has  been  termed  the  "  sustained 
knee-jerk." 

The  sphincters  are  usually  unaffected. 

Diagnosis. — The  irregularity  and  persistence  of  the 
movements  serve  to  distinguish  chorea  from  the  tics. 
In  the  tics,  periods  of  complete  repose  alternate  with 
exacerbations  (p.  468). 

*  Cxreoet  and  Loubet,  op.  cit. 


464    DISEASES   OF   THE   NERVOUS   SYSTEM 

The  athetosis  of  hemiplegia  or  diplegia  in  children 
is  a  much  slower  movement,  and,  moreover,  the  per- 
sistence of  the  movements  and  the  permanent  paralysis, 
which  is  often  accompanied  by  arrested  mental  develop- 
ment and  epilepsy,  all  point  to  the  nature  of  the  case. 
The  history  is  also  frequently  an  aid  in  diagnosis,  for 
in  most  cases  it  is  found  that  the  paralysis  (if  not  the 
athetosis)  and  the  mental  changes  date  from  birth, 
or  have  supervened  shortly  after  at  an  age  at  which 
chorea  never  occurs. 

Hysterical  movements  may  closely  resemble  chorea, 
but,  like  those  of  the  tics,  they  are  usually  more  regular. 
In  cases  of  hysteria  other  confirmatory  manifestations 
will  usually  be  found,  but  it  must  not  be  forgotten 
that  it  is  possible  for  both  conditions  to  be  present  at 
the  same  time. 

In  myoclonus  the  spasms  are  shock-like.  The  trunk 
is  more  liable  to  be  affected,  and  the  contractions  tend 
to  be  symmetrical. 

Prognosis. — Patients  with  rheumatic  chorea  gen- 
erally tend  to  get  well  in  the  course  of  a  few  weeks, 
but  the  progress  is  often  slow,  the  affection  lasting,  on 
an  average,  between  four  and  twelve  weeks.  There  is 
a  strong  tendency  for  the  patient  to  relapse  just  when 
the  symptoms  seem  to  be  abating,  and  there  is  also  a 
distinct  tendency  to  recurrence  after  varying  intervals. 

Exceptions  to  this  benign  course  occur  every  now 
and  then.  Severe  cases  in  which  the  body  is  incessantly 
thrown  about  may  end  fatally  with  symptoms  of  exhaus- 
tion, delirium,  and  hyperpyrexia,  and  to  such  the  term 
chorea  gravis  is  applied. 

Endocarditis  which  develops  during  the  course  of 
chorea  has  the  same  significance  as  that  developing 
during  the  course  of  rheumatic  fever  and  ether  diseases. 

Treatment. — The  main  objects  of  treatment  are  : 

1.  To  obtain  rest  (mental  and  physical). 

2.  To  improve  nutrition. 

3.  To  give  such  drugs  as  may  be  thought  to  have 

a  special  influence  on  the  disorder. 


CHOKEA:    TREATMENT  465 

1.  Rest. — In  cases  of  any  severity  the  child  sliould 
be  kept  in  bed.  In  others  the  time  should  be  spent 
quietly,  free  from  all  excitement,  and  when  possible  it 
is  desirable  to  avoid  the  society  of  other  children. 

2.  Nutrition  must  be  improved  by  a  diet  contain- 
ing plenty  of  milk ;  preparations  of  cod-liver  oil,  iron, 
phosphates,  and  other  similar  tonics  are  very  useful. 

3.  Drugs. — The  fact  that  so  many  drugs  are  recom- 
mended for  the  treatment  of  chorea  is  strong  evidence 
that  no  single  one  can  be  depended  upon  to  cure  it. 

The  drug  that  has  gained  most  popularity  is  arsenic, 
either  given  in  rapidly  increasing  doses  over  a  short 
period  of  time,  or  in  smaller  amounts  over  a  longer  one. 
In  the  first  instance,  where  the  dose  begins  with  2  or  3 
minims  of  liquor  arsenicalis  and  is,  perhaps,  quickly 
raised  to  8  or  10  minims,  careful  observation  must  be 
kept  for  the  appearance  of  any  symptoms  of  acute 
arsenical  poisoning ;  while  in  giving  the  drug  in  smaller 
doses  over  a  longer  period  of  time  there  is  always  a 
danger  of  producing  peripheral  neuritis.  As  in  other 
circumstances,  arsenical  preparations  should  be  well 
dilated  and  given  after  meals. 

The  benefit  to  be  obtained  from  this  form  of  treat- 
ment is  by  no  means  assured,  and  it  is  now  generally 
agreed  that  arsenic  has  no  specific  action  on  the  course 
of  the  disease. 

Salicylates  and  aspirin  are  extolled  by  some,  but, 

in  spite  of  the  close  relationship  of  chorea  to  rheumatism, 

there  is  no  consensus  of  opinion  in  favour  of  these  drugs. 

Phenacetin,  antipyrin,  and  chloretone  are  regarded 

as  useful. 

In  acute  cases,  chloral  hydrate  and  the  bromides 
may  be  required  to  induce  sleep. 

As  a  general  rule,  rest,  good  food,  with  cod-liver  oil  and 
iron  or  some  other  suitable  tonic,  should  form  the  basis  of 
treatment,  while  arsenic,  salicylates,  phenacetin,  or  other 
drugs  may  be  used  additionally  as  occasion  demands. 

Besides  treatment   by  drugs,  the   neurotic    disposi- 
tion of  the  child  needs  careful  consideration,   and  the 
2e 


466    DISEASES   OF   THE  NERVOUS  SYSTEM 

induction  of  happiness  and  contentment  often  materially 
assists  the  cure. 

Leonard  Guthrie  found  that  suggestion  combined 
with  passive  movements,  the  performance  of  voluntary 
movements  under  guidance,  and,  in  the  later  stages,  cor- 
rection of  incoordination  by  means  of  graduated  exercises, 
are  all  useful  adjuncts  to  other  methods  of  treatment. 

Huntington's  Chorea 

This  is  an  hereditary  form  of  chorea,  and  bears  no 
relation  whatever  to  rheumatic  chorea.  It  can  often 
be  traced  through  several  generations ;  both  sexes  suffer, 
but  it  is  said  never  to  skip  a  generation,  so  that  once 
the  tendency  is  broken  it  does  not  reappear. 

The  symptoms  usually  begin  after  middle  age,  and 
consist  of  choreic  movements  and  ataxy  together  with 
mental  degeneration.  The  movements  cease  during 
rest,  but  are  otherwise  very  persistent ;  they  are  slower 
than  those  of  rheumatic  chorea. 

Ataxy  is  a  prominent  feature,  so  that  there  is  often 
a  staggering  gait  like  that  of  a  drunken  man. 

The  mental  changes  are  chiefly  characterized  by 
progressive  enfeeblement. 

The  disease  often  lasts  a  number  of  years.  No 
adequate  treatment  is  known. 

Senile  Chorea 
Occasionally  in  late  middle  life  cases  occur  which 
resemble  in  many  respects  those  of  Huntington's  chorea, 
but  in  these  senile  cases  no  other  members  of  the  family 
suffer.     The  prognosis  is  not  good. 

Electrical  Chorea 
Electrical  chorea  is  the  term  applied  to  a  rare  con- 
dition in  which  sudden  movements  like  those  from  an 
electric  shock  are  associated  with  wasting  of  muscles 
and  pyrexia.  This  disease,  which  progresses  to  a  fatal 
termination,  was  first  described  by  Dubini,  and  appears 
to  occur  in  Italy  only.  The  cause  has  been  ascribed  to 
some  infective  process. 


'^3 


a  eg 

11 


OQ 


CHAPTER  XLIX 

THE  TICS 

The  tics  are  spasmodic  movements  of  psychical  origin 
which  are  repeated  time  after  time,  without  any  definite 
cause  or  object,  and  are,  for  the  most  part,  varieties  of 
the  compulsion  neuroses  {see  p.  498). 

They  usually  represent  voluntary  movements  which 
would  be  useful  in  their  proper  place,  and  their  origin  can 
often  be  traced  to  the  repetition  of  movements  which 
were  at  one  time  performed  with  a  definite  purpose. 
In  this  way  persistent  blinking  may  result  from  errors 
of  refraction  or  injury  to  the  eye  ;  sniffing,  from  nasal 
affection  ;  and  shrugging  the  shoulders,  from  ill-fitting 
clothes.  Normally,  all  such  movements  should  cease 
when  the  causes  which  evoked  them  are  removed,  but 
in  neurotic  or  debilitated  people  they  may  become  a 
habit  and  persist  indefinitely.  Several  different  move- 
ments may  be  out  of  hand  at  the  same  time,  such  as 
blinking  the  eyes  and  twitching  the  mouth  muscles. 

These  tics  must  not  be  confused  with  the  spasms 
which  sometimes  accompany  severe  attacks  of  trigeminal 
neuralgia,  and  which  have  received  the  name  of  tic 
douloureux. 

The  varieties  of  tics  are  innumerable.  When  the 
movements  are  confined  to  a  small  group  of  muscles, 
e.g.  in  blinking  or  sniffing,  they  are  often  called  simple 
tics  or  habit  spasms,  while  the  more  complex  ones  are 
designated  as  coordinate  tics  (Plate  23),  though  all  true 
tics  are,  as  a  matter  of  fact,  coordinate  movements. 

There  are  also  mental  tics,  in  which  an  idea  or  a  tune 
constantly  repeats  itself,  though  here,  again,  it  must 

467 


468    DISEASES   OF   THE   NERVOUS   SYSTEM 

be  understood  that  there  is  a  psychical  element  pre- 
sent in  the  production  of  all  tics.  Tics  of  speech  also 
occur  and  may  coexist  with  impulsive  movements  of 
the  body  ;  in  some  of  these  the  patient  repeats  some 
words  which  he  has  just  heard  spoken  (echolalia), 
and  in  others  blasphemous  or  obscene  expressions  are 
habitually  involuntarily  uttered  (coprolalia).  Latah,  or 
the  jumping  disease,  found  especially  among  Malays,  is 
probably  a  complicated  form  of  tic. 

Etiology. — Allusion  has  already  been  made  to  the 
origin  of  tics  from  movements  which  were  at  first  per- 
formed with  a  definite  object.  Heredity  and  a  neurotic 
temperament  are  both  important  conditions,  as  also 
is  mimicry.  While  tics  of  slight  severity  often  arise 
from  overwork  or  general  debility,  and  disappear  again 
as  the  health  improves,  many  are  very  persistent,  and 
depend  for  their  origin  on  disturbances  of  the  mental 
equilibrium  as  described  in  connection  with  the  psycho- 
neuroses  (p.  479). 

Character  of  the  movements. — The  movements 
constituting  the  tics  usually  consist  of  short,  sharp 
spasms,  which  may  be  executed  with  great  rapidity, 
and' which  have  been  compared  to  the  movements  from 
an  electric  shock  ;  or  they  may  be  more  deliberately 
performed  with  a  rhythm  that  seems  quite  voluntary, 
while  occasionally  they  assume  a  tonic  character.  The 
rapid,  lightning-like  jerks  are  generally  well  seen  in  the 
facial  tics. 

The  performance  of  a  tic  is  usually  preceded  by  a 
desire  to  make  the  movement,  and  followed  by  a  sense  of 
annoyance  at  having  made  it,  but  after  a  time  the 
movement  may  become  so  habitual  that  it  takes  place 
unconsciously. 

The  spasms  are  apt  to  occur  in  bouts,  with  inter- 
missions of  varying  length.  Any  attempt  to  control 
the  movements  involves  great  effort,  which  can  only 
be  sustained  a  very  short  time,  and  is  usually  followed 
by  a  specially  severe  attack.  The  ticS;  as  a  general  rule, 
disappear  during  sleep. 


THE   TICS:    DIAGNOSIS  469 

Diagnosis. — In  children  the  movements  are  liable 
to  be  mistaken  for  those  of  chorea. 

The  distinction  can  be  made  by  noting  that — 

1.  The  same  movement  or  movements  are  constantly 
repeated  in  tics,  whereas  in  chorea  the  movements  are 
irregular  and  various. 

2.  The  movements  in  tics  have  a  more  distinct, 
purposeful  character,  though  they  may  have  no  adequate 
cause  and  bear  no  definite  result.  Those  of  chorea  are 
irregular  and  in  no  way  suggestive  of  purpose. 

3.  In  the  tics  there  are  definite  bouts,  with  intervals 
of  complete  freedom.  In  chorea  there  are  exacerba- 
tions, but  the  intervals  are  occupied  by  movements, 
even  though  slight,  so  that  there  is  not  the  complete 
intermission  that  is  present  in  the  tics. 

From  reflex  s'pasms. — It  is  very  important  to  distin- 
guish between  spasms  due  to  reflex  irritation  and  those 
of  cortical  origin,  which  constitute  the  tics.  Thus,  as 
already  mentioned,  tic  douloureux,  a  reflex  spasm 
arising  from  the  irritability  of  the  5th  nerve  in  severe 
neuralgia,  has  nothing  of  the  characters  of  the  true  tics. 

Reflex  spasms  of  the  sterno-mastoid,  trapezius,  and 
other  muscles  of  the  neck  may  all  give  rise  to  torti- 
collis, and  must  be  carefully  distinguished  from  tics  of 
cortical  origin.  A  physiological  as  opposed  to  an  anato- 
mical distribution  of  the  movements  will  often  be  of  some 
assistance.  For  instance,  a  coordinated  action  between 
difierent  groups  of  muscles,  such  as  the  sterno-mastoid 
on  the  one  side  and  the  deep  rotators  of  the  neck  on  the 
other,  is  much  more  suggestive  of  cortical  origin  than 
of  some  local  nerve  trouble  that  has  by  coincidence 
happened  to  irritate  two  widely  separated  muscles  which 
physiologically  tend  to  act  together. 

Prognosis. — The  outlook  varies  considerably  for  dif- 
ferent cases.  In  children,  simple  tics  often  entirely 
disappear,  but  in  adults  the  result  is  not  so  hopeful. 

Those  which  have  arisen  during  a  period  of  special 
strain  are  more  likely  to  disappear  in  favourable  cir- 
cumstances than  those  that  have  come  on  insidiouslv 


470    DISEASES  OF  THE  NERVOUS   SYSTEM 

during  the  ordinary  daily  life.  In  many  the  movements 
diminish  with  improvement  in  the  general  health,  but 
are  aj^t  to  show  themselves  again  very  readily  during 
worry  or  excitement. 

Treatment. — ^Any  source  of  peripheral  irritation 
which  causes  or  aggravates  the  spasm  must  be  removed  ; 
next,  the  general  health  must  be  attended  to.  Elec- 
tricity is  useful,  but  needs  care.  Where  the  movement 
tends  to  assume  a  tonic  character,  as  in  some  of  the 
cases  of  torticollis,  faradism  of  the  opposite  muscles  is 
sometimes  beneficial,  while  galvanism  may  be  applied 
to  those  at  fault. 

Carefully  graduated  exercises,  whereby  the  patient 
learns  to  arrest  some  movements  and  to  make  others 
more  regular,  are  often  very  useful,  and  may  be  practised 
in  front  of  a  looking-glass.  A  course  of  psychothera- 
peutics, which  may  be  combined  with  massage  and 
general  exercises,  will  often  be  found  beneficial  in 
selected  cases. 

The  treatment  of  some  forms  of  localized  tics  by 
surgical  measures  is  tempting,  but  must  always  be 
approached  with  great  caution.  Only  very  exception- 
ally are  these  measures  appropriate  ;  the  recollection 
of  this  will  often  avert  the  disappointment  of  finding 
the  spasm  extending  to  other  muscles  after  the  nerves 
to  those  originally  affected  have  been  cut.  On  the 
other  hand,  the  spasms  of  reflex  origin  may  often  be 
more  favourable  for  surgical  intervention. 

Nodding  Spasms  (Spasmus  Nutans) 
Ehythmical  movements  of  the  head,  either  of  a 
nodding  or  of  a  side-to-side  character,  associated  with 
a  lateral  or  rotatory  nystagmus,  are  frequently  met  with 
in  infants.  The  origin  is  not  clear,  but  the  usual  asso- 
ciated conditions  are  debility,  teething,  or  rickets. 

The  prognosis  is  good,  and  in  most  of  the  cases  the 
abnormal  movements,  both  of  head  and  of  eyes,  cease  as 
the  child  grows  older.  Cod-liver  oil  and  other  tonics  are 
useful  in  treatment; 


CHAPTEli  L 

MYOCLONUS;    MYOTONIA  CONGENITA;    FAMILY 
PERIODIC  PARALYSIS 

MYOCLONUS  (PAEAMYOCLONUS) 

Myoclonus  is  characterized  by  rapid,  sudden  con- 
tractions of  muscles  resembling  those  obtained  by  an 
electric  shocks 

Etiology. — ^Nothing  definite  is  known  about  its 
causation  beyond  the  fact  that  debility  and  ill-health 
seem  often  to  have  preceded  it.  It  sometimes  occurs  in 
several  members  of  the  same  family,  and  it  is  apt  to  be 
associated  with  epilepsy. 

Symptoms.  —  The  characteristic  symptoms  are 
repeated,  sudden,  electric-shock-like  contractions  of 
muscles,  chiefly  of  the  limbs,  less  often  of  the  trunk, 
and  least  often  of  the  face. 

The  contractions  are  usually  bilateral,  and  occur 
on  both  sides  either  at  the  same  time  or  immediately 
after  one  another.  If  they  are  sufiiciently  strong  and 
extensive  the  whole  limb  moves,  or  in  severe  cases  the 
patient  may  be  thrown  down.  The  spasms  usually  cease 
during  sleep,  and  are  often  ameliorated  during  voluntary 
movements.  The  reflexes  are  generally  exaggerated,  and 
the  nutrition  of  the  muscles  remains  good. 

The  seat  of  the  disease  is  uncertain,  but  from  its 
occasional  association  with  epilepsy  the  cerebral  cortex 
seems  to  be  the  most  likely  place. 

Diagnosis. — The  movements  differ  from  those  of 
rheumatic  chorea  in  their  suddenness,  persistence,  and 
distribution,  while  there  are  not  the  wasting  and  general 

471 


472    DISEASES   OF   THE  NEEVOUS   SYSTEM 

symptoms  met  with  in  electrical  chorea.  The  tics  may- 
be confused  with  myoclonus,  but  they  are  more  purposive 
and  more  likely  to  be  one-sided. 

Prognosis. — There  is  no  certainty  about  the  course 
of  these  cases.  In  most  of  them  the  movements  persist, 
but  in  a  fcAV  they  cease  spontaneously  or  as  the  result  of 
treatment. 

<•  Treatment. — In  the  absence  of  any  discoverable 
source  of  irritation,  the  treatment  must  be  conducted 
on  general  hygienic  principles.  If  the  spasms  are  very 
violent,  sedatives,  such  as  hyoscyamus,  may  be  needed, 
and  if  epilepsy  is  present  the  bromides  should  be  given. 

MYOTONIA   CONGENITA   (THOMSEN'S 
DISEASE) 

Etiology. — This  disease  appears  to  be  due  to  some 
deficiency  in  development,  and  the  symptoms  generally 
appear  during  childhood.  It  is  essentially  a  family 
disease,  and  is  frequently  handed  on  through  several 
generations. 

Pathology.  —  Some  hypertrophy  of  the  muscles 
often  occurs,  but  there  do  not  appear  to  be  any  changes 
in  the  central  nervous  system.  It  seems  possible  that 
this  is  one  of  the  conditions  which  may  be  at  any  rate 
partially  explained  by  a  want  of  proportion  in  or  modi- 
fication of  the  two  main  constituents  of  muscle  fibre 
(p.  42),  so  that  the  power  of  the  substance  causing  tonic 
contractions  becomes  the  dominating  feature. 

Symptoms. — This  disease  is  characterized  by  the 
occurrence  of  spasms  at  the  beginning  of  a  voluntary 
movement,  which  gradually  pass  off  with  the  persistence 
of  the  movement. 

On  attempting  to  walk  after  resting,  the  legs  are  stiff 
and  can  at  first  only  be  moved  with  difficulty ;  when 
the  movement  is  once  established  they  become  supple, 
and  remain  so  until  after  another  interval  of  rest. 

The  difficulty  in  starting  a  movement  is  well  seen 
by  asking  the  patient  to  open  and  shut  his  eyes  or  his 
hands. 


FAMILY   PERIODIC    PARALYSIS  473 

The  muscles  are  usually  well  developed  and  even 
hypertrophied,  thougli  there  is  often  some  degree  of 
weakness  to  be  detected. 

Electrical  reactions. — The  reaction  of  the  muscles 
is  difi'erent  from  normal  to  both  the  faradic  and  constant 
currents.  With  the  faradic  current  a  longer,  more 
tetanic  contraction  than  usual  is  obtained.  To  the 
galvanic  current  the  muscles  are  unduly  excitable,  and 
the  strength  of  the  contraction  obtained  by  the  closure 
of  the  anode  (ACC)  approaches  or  becomes  equal  to  that 
of  the  kathode  (KCC).  Direct  percussion  of  the  muscles 
also  causes  a  slow  contraction. 

Prognosis  and  treatment. — The  disease  does 
not  shorten  Hfe. 

The  only  form  of  treatment  which  appears  to  produce 
any  benefit  is  that  of  gymnastic  exercises. 

FAMILY  PERIODIC  PARALYSIS 

This  disease,  as  the  name  implies,  runs  in  families 
and  is  also  transmitted  from  one  generation  to  another. 
It  occurs  in  both  sexes. 

Etiology. — The  family  and  hereditary  distribution 
is  suggestive  of  some  developmental  disorder,  while 
the  periodic  nature  of  the  attacks  seems  to  point  to  the 
likelihood  of  a  temporary  toxaemia. 

Excessive  fatigue,  specific  fevers,  constipation,  and 
indigestion  are  among  the  conditions  that  are  thought 
to  dispose  to  the  onset  of  the  attacks. 

Symptoms.  —  The  characteristic  symptoms  are 
temporary  paralysis  with  loss  of  reflexes  and  electrical 
reactions. 

The  attacks  occur  from  time  to  time  after  intervals 
of  various  lengths.  The  onset  is  generally  during  the 
night,  the  patient  waking  up  to  find  that  he  cannot 
move  his  limbs.  Observation  has  shown  that  power 
is  generally  lost  in  the  legs  first,  and  later  in  the  arms 
and  trunk.  The  muscles  suppHed  by  the  cranial  nerves 
are  occasionally  weakened,  in  which  case  there  may  be 
symptoms  of  facial  and  bulbar  paralysis. 


474    DISEASES  OF  THE  NERVOUS  SYSTEM 

The  paralysis  is  of  a  flaccid  character,  and  the  deep 
reflexes  and  electrical  reactions  in  the  paralysed  muscles 
are  diminished  or  even  lost. 

The  heart  may  be  dilated  and  irregular  in  its  action 
during  the  attacks. 

Respiration  is  impaired  by  the  paralysis  extending 
to  the  trunk  muscles,  but  it  is  kept  up  by  the  diaphragm, 
which,  fortunately,  appears  but  seldom  to  be  seriously 
affected. 

Sensation  and  consciousness  are  not  impaired. 

After  some  hours,  power  begins  to  return  in  the  limbs 
id  the  reverse  order  to  that  in  which  they  were  affected. 

Prognosis. — The  outlook,  so  far  as  life  is  concerned, 
is  usually  good,  but  occasionally  death  occurs  during 
an  attack. 

Treatment. — On  the  assumption  that  some  poison 
is  present,  the  diet  should  be  plain  and  the  excretory 
organs  should  be  kept  active.  Diuretics,  especially  citrate 
of  potash,  have  been  recommended  for  shortening  and 
lightening  the  severity  of  the  attacks. 

No  constant  results  seem  to  follow  the  administra- 
tion of  drugs,  but,  of  course,  strychnine,  digitalis,  and 
others  are  very  necessary  to  assist  a  failing  heart  or  an 
enfeebled  respiration,  and  in  the  case  of  the  latter  it 
may  be  necessary  to  give  oxygen  and  to  perform  artificial 
respiration  as  well. 


CHAPTER  LI 

OCCUPATION    NEUROSES 

All  occupations  which  necessitate  for  their  perform- 
ance a  constant  repetition  of  exactly  the  same  move- 
ments are  liable,  after  a  varying  length  of  time,  to 
become  difficult  or  impossible  to  carry  out.  As  a  general 
rule,  the  more  delicate  and  accurate  the  repeated  move- 
ments require  to  be,  the  more  Ukely  is  the  power  of 
performing  them  to  be  lost,  especially  if  constant  con- 
centration and  mental  strain  be  required  at  the  same 
time ;  and,  further,  individuals  who  are  naturally  of 
nervous  temperament  are  probably  the  most  likely  to 
sufier  this  loss  of  power. 

Among  those  especially  liable  to  be  troubled  in  this 
way  are  writers,  piano  and  violin  players,  typists,  and 
telegraphists ;  the  afiection  is  also  met  with  in  workers 
in  many  other  occupations,  such  as  seamstresses,  cigarette 
makers,  and  dairymaids.  One  of  the  most  interesting 
cases  that  have  come  under  the  writer's  notice  was  that  of 
a  man  whose  chief  business  was  to  sharpen  razors,  and 
who  was  unable  to  carry  the  blade  smoothly  backwards 
and  forwards  on  the  stone  on  account  of  sudden  little 
spasms  of  the  arm  which  quite  spoilt  the  accuracy  of 
the  movement. 

Writer's  cramp,  which  is  one  of  the  most  familiar 
of  occupation  neuroses,  occurs  mostly  in  those  who 
have  to  write  a  stiff,  copy-book  hand  for  many  hours  a 
day.  It  is  distinctly  connected  with  a  prolonged  cramped 
movement,  for  it  does  not  seem  to  occur  in  those  who 
write   a   free  hand   from  the  shoulder.     Clerks  whose 

475 


476    DISEASES   OF  THE  NERVOUS  SYSTEM 

chief  business  it  is  to  be  continually  copying  long  docu- 
ments are  among  the  most  common  sufferers. 

Symptoms. — The  symptoms  are  mainly  those  of 
cramp  in  the  small  muscles  of  the  hand.  After  writing 
for  a  longer  or  shorter  time,  according  to  the  severity 
of  the  case,  the  fingers  become  stiff  and  involuntarily 
separate  themselves,  so  that  it  is  impossible  to  hold  the 
pen,  which  is  spasmodically  dragged  across  the  paper 
or  even  shot  out  of  the  hand ;  in  some  cases  there  is, 
every  now  and  then,  a  strong  flexor  spasm. 


«-^ 


Fig.  113.— Specimen  of  handwriting  in  writer's  cramp. 

Pain  is  a  variable  symptom,  but  there  is  often  dis- 
comfort, with  tingling  and  aching  sensations.  In  some 
cases  weakness  is  a  more  prominent  feature  than  spasm. 

At  first  the  patient  finds  he  cannot  write  so  long  or 
so  quickly  as  he  could  formerly,  and  then  the  difficulty 
becomes  greater  and  greater  until  at  last  his  occupation 
is  gone. 

Fig.  114.— Specimen  of  former  handwriting  from  the  same 
case  as  in  Fig.  113. 

The  accompanying  figures  (Figs.  113,  114)  show  how 
the  style  of  writing  may  be  affected. 

Most  movements  other  than  those  concerned  in 
writing  can  often  be  carried  out  with  care,  but  occa- 
sionally some  of  the  finer  ones  suffer  a  little,  although 
usually  not  to  anything  like  the  same  degree. 

The  electrical  reactions  are  often  unchanged.  In 
severe  cases  the  muscles  are  said  to  be  over-excitable  ; 
more  rarely  the  reactions  are  diminished. 

Seat  of  the  lesion. — The  cerebral  cortex,  the 
peripheral  nerves,  and  the  muscles  themselves  have  all 
been  regarded   as   at   fault,  but  it  is   difficult  to  see 


WKITER'S    CRAMP  477 

how  a  change  in  a  peripheral  nerve  can  afiect  one 
set  of  movements  to  the  exclusion  of  others.  Nor 
is  there  any  good  evidence  that  the  fault  lies  in  the 
muscles. 

The  hypothesis  most  in  harmony  with  the  facts  is 
that  the  trouble  is  of  cerebral  origin  and  due  presumably 
to  incoordination  following  exhaustion  of  the  particular 
combination  of  nerve  cells  from  which  the  movement 
originates.  "  The  education  of  centres  which  may  be 
widely  separated  from  each  other  for  the  performance 
of  any  delicate  movement  is  mainly  accomplished 
by  lessening  the  lines  of  resistance  between  them, 
so  that  the  movement,  which  was  at  first  produced 
by  a  considerable  mental  effort,  is  at  last  executed 
almost  unconsciously.  If,  therefore,  through  prolonged 
excitation,  this  lessened  resistance  be  carried  too  far, 
there  is  an  increase  and  irregular  discharge  of  nerve 
energy,  which  gives  rise  to  spasm  and  disordered  move- 
ment. According  to  this  view,  the  muscular  weakness 
is  explained  by  an  impairment  of  nutrition  accompanying 
that  of  function,  and  the  diminished  faradic  excitability 
by  the  nutritional  disturbance  descending  the  motor 
nerves." — Gay  (quoted  by  Osier). 

In  other  instances  the  loss  of  movement  is  not  so 
strictly  limited,  and  pain  along  the  course  of  a  nerve, 
with  perhaps  some  slight  evidences  of  a  muscular  wasting 
and  changes  in  electrical  reaction,  seems  to  point  to 
the  possibility  that  the  trouble  is  connected  with  the 
peripheral  nerves. 

Diagnosis. — When  a  patient  complains  of  difficulty 
in  writing,  it  is  necessary  to  exclude  other  possible 
causes  before  concluding  that  it  is  a  case  of  writer's 
cramp. 

1.  Examine  the  hand  and  wrist  for  any  local  trouble 
such  as  teno-synovitis. 

2.  Ascertain  if  there  are  any  signs  of  localized 
neuritis,  as  shown  by  weakness  in  all  movements,  pain 
and  Avasting,  or  of  multiple  neuritis  with  a  tendency 
towards  a  symmetrical  distribution. 


478    DISEASES   OF   THE   NERVOUS   SYSTEM 

3.  Remember  that  tremor  from  any  cause  will  spoil 
the  writing ;  and  bear  in  mind,  among  other  things,  the 
possibility  of  commencing  paralysis  agitans,  disseminated 
sclerosis,  and  general  paralysis  of  the  insane. 

Prognosis. — The  prognosis  is  not  good,  so  far  as 
the  possibility  of  recovering  power  to  perform  the 
particular  movement  is  concerned.  It  is  only  seldom 
indeed  that  anyone  who  develops  an  occupation  neurosis 
to  an  advanced  degree  is  ever  able  to  earn  his  living 
again  by  that  particular  movement,  but  by  taking 
trouble  and  assisting  with  the  other  hand  the  patient  is 
often  able  to  continue  for  a  considerable  time  before 
having  to  give  up.  Cases  in  which  the  lesion  seems 
to  be  in  the  periphery  are  probably  more  favourable 
than  those  of  cortical  origin. 

Treatment. — Complete  rest  from  the  particular 
occupation  at  the  very  first  sign  of  the  trouble  gives 
the  best  chance  of  ultimate  recovery.  Occupations 
involving  other  and  coarser  movements  may  be  con- 
tinued, provided  that  care  is  taken  to  avoid  tiring  the 
affected  limb.  Locally,  massage,  hydropathic  treat- 
ment, and  galvanism  are  all  beneficial.  The  faradic 
current  should  generally  be  avoided. 

If  the  pain  and  spasm  persist  when  the  occupation 
has  been  left  off,  such  drugs  as  bromides,  phenacetin, 
and  antipyrin  may  be  given,  but  the  main  effort  in 
general  treatment  should  usually  be  towards  improving 
the  health  by  rest  and  a  nutritious  diet  with  plenty  of 
milk  and,  if  needful,  cod-liver  oil.  Various  devices 
are  usually  adopted  by  the  sufferers  to  put  off  the  evil 
day  of  relinquishing  their  work.  In  writer's  cramp  the 
patient  finds  he  can  get  along  better  for  a  time  by 
holding  the  pen  between  the  first  and  second  fingers, 
and  from  this  he  takes  to  a  very  thick  penholder,  which 
he  grasps  with  the  whole  hand.  Such  devices,  however, 
can  only  temporarily  hide  an  irremediable  deterioration. 


CHAPTEE  LII 

PSYCHONEUROSES 

GENERAL    INTRODUCTION 

The  Psychoneuroses  comprise  a  group  of  cases  in  which 
the  symptoms  represent  some  degree  of  failure  of  the 
indi\adual  to  adapt  himself  adequately  to  the  realities 
of  his  daily  life.  Among  the  principal  varieties  are 
Hysteria,  Compulsion  Neuroses,  Neurasthenia,  and 
Anxiety  Neuroses. 

A  nervous  breakdown  is,  generally  speaking,  due 
to  a  disturbance  of  equilibrium  between  the  adapta- 
bility of  the  individual  and  the  complexity  of  his  sur- 
roundings. In  one  case  the  power  of  adaptation  may 
be  abnormally  weak,  while  in  another  the  difficulties 
to  be  overcome  may  be  unusually  great.  It  is  on  the 
relative  strength  of  these  two  factors  that  the  resulting 
type  of  nervous  reaction  depends,  as  evidenced  by  the 
strength  or  weakness  with  which  people  respond  to  the 
ordinary  difficulties  of  daily  life.  That  defective  power 
of  adaptation,  which  may  be  either  inherited  or  ac- 
quired, is  of  great  importance  has  been  shown  by  con- 
sideration of  "  shell  shock  "  cases,  the  histories  of  which 
frequently  revealed  a  previous  breakdown  owing  to 
inability  to  meet  the  requirements  of  even  the  ordinary 
conditions  of  civil  life. 

The  symptoms  are  caused  by  a  disturbance  of  the 
contents  of  the  mind,  which  contents,  according  to 
modern  conceptions  of  psychology,  are  to  be  considered 
under  the  aspects  of  the  "  conscious,"  the  "  subcon- 
3cious,"  and  the  "  unconscious  "  mind. 

479 


480    DISEASES   OF   THE  NERVOUS   SYSTEM 

The  "  conscious  mind  "  represents  all  those  thoughts 
which  are  known  to  the  person  at  any  given  time. 

The  *'  subconscious  (preconscious  or  foreconscious) 
mind  "  is  concerned  with  ideas  which,  though  not  in  the 
field  of  consciousness  at  a  given  moment,  can  easily  be 
called  up  by  the  processes  of  association  of  ideas. 

The  "  unconscious  mind  "  contains  memories  and 
ideas  which  can  only  be  called  into  consciousness  with 
great  difficulty,  usually  by  the  aid  of  such  methods  as 
hypnotism  or  the  free  association  of  psychoanalysis. 

According  to  this  concejotion,  there  is  free  inter- 
change always  taking  jDlace  between  the  ideas  of  the 
conscious  and  the  subconscious  levels,  but  very  little,  if 
any,  between  those  of  the  conscious  and  the  unconscious 
levels  ;  and  it  is  in  the  unconscious  plane  that  memories 
of  a  painful  nature  and  ideas  representing  such  broad 
primitive  desires  as  self-preservation  and  race-preserva- 
tion are  repressed  when  full  gratification  of  such  desires 
is  incompatible  with  recognized  social  conduct. 

On  this  hypothesis,  symptoms  of  "  nervousness " 
may  be  brought  about  mainly  in  two  ways  : — 

(1)  By  the   reproduction,   through  processes   of 

mental  association,  of  the  emotional  state 
corresponding  to  a  painful  memory,  the 
nature  of  the  latter  often  being  unrecognized 
by  the  patient. 

(2)  By    a    mental    conflict    resulting    from    the 

desire  to  attain  ends  which  may  be  in  a 
large  degree  incompatible  with  the  conven- 
tions of  ordinary  life. 

A  simple  example  of  the  first  type  is  given  by  Rows,* 
of  a  man  who  could  ride  in  a  bus  or  train  without 
discomfort  but  who  fell  into  a  state  of  terror  if  he 
entered  a  tram.  On  examination  of  his  mental  state 
the  disability  was  found  to  be  caused  by  the  sound  of 
the  trolly  wheel  on  the  wire,  which  was  unconsciously 
associated  in  his  mind  with  the  sound  of  an  approaching 

*  Morison  licctures,  1920,     li^din.  Med.  Journ, 


PSYCHONEUROSES  481 

shell  that  had  broken  into  his  dug-out,  with  resulting 
injury  to  himself  and  loss  of  life  to  some  of  his 
companions. 

In  the  second  grouj),  if  the  conflict  between  the 
suppressed  desires  and  their  possibilities  of  attainment 
cannot  be  settled  satisfactorily  by  working  off  the  energy 
in  a  useful  manner,  i.e.  in  the  activities  of  everyday 
life,  the  result  may  be  the  formation  of  some  kind  of 
neurosis  which  may  take  various  forms,  e.g.  an  anxiety 
neurosis,  a  comjDulsion  neurosis  such  as  a  tic,  or  a  so- 
called  conversion  neurosis,  in  which  peace  of  mind  is 
sought  at  the  expense  of  physical  disability,  as  occurs 
in  cases  of  hysterical  paralysis. 

On  the  bodily  side  any  conditions  which  impair  the 
nutrition  of  the  nervous  system,  as  fatigue  (mental  or 
physical),  injury,  toxaemias,  arterio-sclerosis,  will  facili- 
tate the  development  of  changes  in  the  mental  state. 

Particular  mention  must  also  be  made  of  the  internal 
secretions  (the  endocrinic  system),  which  recent  re- 
searches have  shown  to  have  an  important  influence 
on  nervous  metabolism  through  the  autonomic  system 
{see  p.  48). 

When  mental  balance  is  upset  in  one  of  these 
ways  a  state  of  '"  nervousness  "  and  "  apprehensive- 
ness  "  is  apt  to  arise,  and  upon  this  state  further 
mental  and  bodily  symptoms  may  develop  according  to 
the  type  of  the  patient  and  the  surroundings  in 
which  he  is  placed. 

The  ease  or  otherwise  with  which  such  a  "  nervous  " 
state  is  brought  about  depends  upon  the  degree  of 
stability  of  the  individual  in  relation  to  the  intensity 
of  the  debilitating  conditions  by  which  he  is  afiected. 

There  are  some  persons  whose  nervous  systems  are 
so  constituted  that  they  quickly  feel  any  undue  stress, 
and  are  unable  to  adjust  themselves  adequately  even 
to  the  average  circumstances  of  daily  life.  Such  people 
are  in  a  more  or  less  chronic  condition  of  nervousness, 
which  varies  according  to  their  state  of  general  health 
and  to  the  surroundings  in  which  they  happen  to  be 
2f 


482    DISEASES   OF   THE  NERVOUS   SYSTEM 

situated.  On  the  other  hand,  those  who  possess  ner- 
vous systems  of  average  or  greater  than  average  stability 
will  only  become  nervous  from  some  unusual  stress  of 
mind  or  body. 

Assuming  the  nervous  symptoms  to  begin  as  the 
result  of  some  shock  or  mental  conflict  as  suggested 
above,  the  sequence  of  events  may  be  broadly  indicated 
as  follows  : — 

Mental  shock  or  contlict. 
Emotion, 


Bodily   disturbance,   mainly       (a)  Mental  disturbance,  feeling 
through  autonomic  system.  of  apprehension,  and  general 

"nervousness." 


(Z*)  Possible  further  development 
of  more  definite  mental 
symptoms,  e.g.  obsessions, 
general  lack  of  perspective, 
suspicion,  hypochondriasis, 
or  delusions. 

The  more  definite  developments  enable  further 
classification  to  be  made,  and  among  the  principal  states 
thus  recognized  are  those  known  as  Neurasthenia, 
Anxiety  Neuroses,  Comjmlsion  Neuroses  and  Hysteria. 
It  must,  however,  be  distinctly  understood  that  no  very 
definite  line  can  be  drawn  between  these  different 
varieties,  for  frequently  there  are  many  symptoms 
common  to  all,  and  the  classification  is  to  be  justified  on 
grounds  of  convenience  rather  than  of  reality. 

HYSTERIA 

Under  the  term  hysteria  are  included  a  variety  of 
well-recognized  symptoms  which  appear  to  depend 
essentially  upon  a  disorder  of  mind. 


HYSTERIA  483 

It  is  obvious  that  symptoms  depending  on  such  a 
cause  cannot  have  any  sharply  defined  boundaries,  for 
many  organic  diseases  are  naturally  often  preceded  or 
accompanied  by  similar  symptoms  due  to  changes  in  the 
irritability  and  conductivity  of  the  nervous  system  before 
any  signs  of  organic  destruction  can  be  detected,  as  also 
are  many  of  the  more  severe  "  functional  diseases," 
among  which  are  included  some  of  the  forms  of  insanity. 

In  the  absence  of  any  structural  change,  the  exact 
nature  of  the  changes,  if  any,  underlying  hysteria  must 
still  be  regarded  as  unknown.  Since  the  time  when  the 
symptoms  were  thought  to  be  caused  by  movements  of 
the  uterus  many  hypotheses  have  been  suggested,  some 
of  which  place  the  disease  on  a  physical  and  others  on  a 
mental  basis.  Among  the  more  important  of  tJtie  views 
put  forward  in  recent  times  are  those  of  Janet,  Babinski, 
and  Freud. 

Janet's  view  is  based  upon  the  phenomena  of  dis- 
sociation of  consciousness.  "  Hysteria,"  he  says,  "  is  a 
form  of  mental  depression,  characterized  by  the  retrac- 
tion of  the  field  of  personal  consciousness  and  a  tendency 
to  the  dissociation  and  emancipation  of  the  systems 
of  ideas  and  functions  that  constitute  personality."  * 

According  to  Babinski,  the  characteristic  of  an 
hysterical  symptom  is  that  it  can  be  produced  by  sug- 
gestion and  made  to  disappear  by  persuasion,  a  condition 
of  things  to  which  he  has  given  the  name  jnthiatism 
(Treidu),  persuasion,  larog,  curable).  On  this  basis  he  con- 
siders hysteria  to  be  a  pathological  condition  showing 
itself  by  a  set  of  primary  symptoms  which,  in  cer- 
tain subjects,  it  is  possible  to  reproduce  exactly  by 
suggestion,  and  which  can  be  made  to  disappear  again 
solely  by  persuasion.  In  this  group  of  primary  symptoms, 
which  have  in  common  the  characteristics  of  being 
reproducible  by  suggestion  and  removable  by  persuasion. 
Babinski  places  convulsions,  paralyses,  contractures, 
tremors,  rhythmical  or  irregular  choreiform  movements, 
and  disturbances  of  sensation,  respiration,  and  phonation. 
*  "Major  Symptoms  of  Hysteria." 


484    DISEASES   OF   THE   NERVOUS   SYSTEM 

There  is,  however,  a  group  of  secondary  symptoms 
which  are  not  directly  dependent  on  suggestion  for  their 
production,  including  exaggeration  of  cutaneous  reflexes, 
tachycardia,  erythemata,  and  excessive  secretion  of  the 
skin  or  the  intestine. 

Freud  considers  that  hysteria  is  the  result  of  painful 
experiences  (mental  traumata),  the  memories  of  which, 
through  their  unpleasantness  to  the  patient,  have  become 
dissociated  from  the  conscious  part  of  the  mind  and 
repressed  into  that  of  the  unconscious.  The  natural  end 
of  such  painful  mental  experiences  is  neutralization  by 
suitable  action  or  gradual  dimming  of  their  effects  by 
time ;  but  when  for  any  reason  the  impressions  are  not 
thus  neutralized,  the  memory  is  apt  to  be  buried  only 
and  not  destroyed,  and  is  able  then  in  certain  circum- 
stances to  resume  its  activity  and  to  influence  the 
feelings  and  activities  of  the  patient  by  clashing  with 
his  general  ideas  of  behaviour.  According  to  these 
views,  the  hysterical  symptoms  (e.g.  paralyses  and 
anaesthesias)  represent  manifestations  of  certain  com- 
plexes that  have  become  dissociated  or  "  split  off  "from 
consciousness  and  form  the  type  known  as  conversion 
hysteria,  in  which  the  mental  trouble  is  got  rid  of  at  the 
expense  of  physical  disability.  In  another  form,  spoken 
of  as  anxiety  hysteria,  the  principal  symptoms  are  appre- 
hension, phobias  and  hysterical  attacks  similar  to  those 
occurring  in  the  group  of  "  anxiety  neuroses."  Space 
will  not  j)ermit  Freud's  theories  to  be  dealt  with  in  further 
detail ;  for  fuller  information  the  reader  should  consult 
special  books  on  the  subject. 

Etiolofjy. — Hysteria  occurs  more  frequently  in 
females  than  in  males,  although  the  latter  are  by  no 
means  exempt ;  quite  young  children  may  also  be  sub- 
jects of  the  disease.  It  is  common  for  the  symptoms 
to  appear  about  the  time  of  puberty  or  soon  after- 
wards. Heredity  is  an  important  factor,  as  also  are  the 
natural  temperaments  of  different  nationalities.  The 
first  symptoms  may  set  in  after  a  mental  or  physical 
shock.     Lastly,  it  must  never  be  forgotten  that  some 


HYSTERIA:    SYMPTOMS  485 

definite  organic  lesion  may  be  associated  witii  the  advent 
of  hysterical  manifestations. 

Symptoms. — The  symptoms  are  so  numerous  and 
ill-defined  that  it  is  very  difficult  to  classify  them  satis- 
factorily, but  some  such  grouping  as  the  following  may 
be  found  useful : 

[  Cnder-action,     Paralysis, 
f  Tremors. 
I.  :ifofor  si/sfcm       ••■  i  Qver-action.    J  ^pasms    and     contrac- 


1      tures. 

\^  Convulsions. 


fUnder-action.     Anaestliesia. 
f  Pain. 
^.  ^^,^cv,y  .,^o.t.,«    •  •  • 'i  Q vGr-action     J  Hyperaistliesia. 

j  Hysterogenelic 
[^  y     points. 

3.  Special  senses        ...     Eyes.       Spasms  of  ocular  muscles  and 

defects  of  visual  power. 
Ears.       Deafness. 
Taste.      Absent  or  modified. 
Smell.     Absent  or  modified. 

4.  Mental  symptoms  .,      Impaired  volition. 

Irritability. 
Depression. 
Exaltation. 
Loss  of  memory. 

The  Motor  System 
under-action 

Paralyses. — The  paralyses  are  principally  found  in 
one  limb  (monoplegia),  in  an  arm  and  a  leg  (hemi- 
plegia), or  in  both  legs  (paraplegia),  from  which  it  is 
easy  to  see  how  closely  they  may  simulate  the  effects 
of  organic  disease.  Of  these  three  varieties  the  para- 
plegic distribution  is  perhaps  the  most  common. 

The  chief  characteristics  of  these  paralyses  are  loss 
of  power  without  wasting  or  any  electrical  changes 
in  the  muscles,  and  frequently  without  definite  altera- 
tions in  the  reflexes. 

The  loss  of  power  varies  in  amount ;  it  may  be  quite 
complete  or  only  moderate  in  degree.  Some  patients 
can  move  the  various  segments  of  a  limb  when  lying 


486    DISEASES   OF   THE  NERVOUS   SYSTEM 

down,   but  are  nevertheless  unable  to  stand  or   walk 
(astasia-abasia). 

Reflexes.  —  The  Jcnee-jerJcs  are  frequently  increased — 
never  absent — and  there  is  often  a  "  pseudo  "  anUe- 
clonus,  which  is  characterized  by  a  few  short  taps, 
generally  ceasing  when  the  foot  is  fully  flexed.  This 
pseudo-clonus,  as  it  is  called,  is  not  of  much  value, 
since  an  incomplete  clonus  is  often  obtained  in  organic 
disease. 

On  the  other  hand,  a  strong  and  sustained  clonus 
is  greatly  in  favour  of  organic  disease,  though  not  by 
itself  necessarily  conclusive. 

Plantar  reflex. — The  reaction  of  the  plantar  reflex  is 
very  important,  for  an  undoubted  extensor  response 
is  an  indication  of  organic  disease. 

In  many  instances  of  functional  disorder  there  are 
times  when  no  plantar  reflex  of  any  kind  can  be  ob- 
tained, and,  although  such  cases  are  suggestive  of 
functional  disorder,  too  much  stress  must  never  be  laid 
on  a  negative  result,  since  experience  shows  that  a 
plantar  reflex  which  is  either  flexor  or  is  not  obtain- 
able on  one  day  may  on  another  give  a  definite  extensor 
reaction. 

One  may  sum  up  the  comparative  importance 
of  the  different  reactions  of  the  plantar  reflex,  as  far 
as  the  diagnosis  of  hysterical  paralysis  is  concerned,  as 
follows  : — 

Extensor  response. — Indicates  the  presence  of 

organic  disease  of  the  motor  tracts. 
Flexor  response. — Suggestive  of  the  absence 
of  organic  disease  of  the  upper  motor  tracts. 
Absence  of  response. — Suggestive  of  functional 
disease  when  taken  with  other  symptoms, 
but  care  must  be  taken  to  see  that  the  ab- 
sence is  not  merely  accidental  or  temporary, 
a  condition  which  frequently  occurs,  e.g. 
from  dampness  or  coldness  of  the  feet.* 

*  The  conditions  in  which  the  plantar  reflex  is  likely  to  be  absent 
are  discussecl  at  p,  30. 


HYSTEEIA:    MOTOR    SYMPTOMS  487 

Sphincters. — There  may  be  retention  of  urine  with 
overflow-incontinence,  but  never  the  true  incontinence 
that  occurs  when  the  lumbar  enlargement  of  the  cord 
is  damaged. 

Larynx. — Paralysis  of  the  laryngeal  movements  is 
common,  and  gives  rise  to  aphonia,  which  often  dis- 
appears as  suddenly  as  it  arises.  It  is  ahvays  the 
adductor  movements  that  are  affected,  so  that  the  cords 
are  widely  separated  and  cannot  voluntarily  be  brought 
together,  although  they  move  fieely  during  respiration 
and  coughing  w^hen  these  acts  are  automatically  per- 
formed without  the  intervention  of  the  will: 

The  paralyses  of  the  larynx  and,  more  rarely,  those 
of  the  face  are  the  only  ones  that  are  likely  to  occur 
in  the  distribution  of  the  cranial  nerves.  The  squints 
and  ptosis  which  may  be  met  with  are  usually  due  to 
spasms,  and  are  referred  to  in  the  section  below,  headed 
Over-action. 

General  summary  of  the  diagnosis  of  hysterical  paralyses. — 
The  procedure  in  diagnosing  hysterical  paralysis  does 
not  differ  from  that  required  in  the  diagnosis  of  loss 
of  power  from  other  causes. 

The  possibilities  must  all  be  carefully  considered, 
and  the  diagnosis  of  hysteria  only  arrived  at  after  rigid 
exclusion  of  everything  else.  Assuming  the  patient  to 
have  lost  fower  in  the  legs,  the  following  questions  must 
be  asked  : 

1.  7s  the  lesion  situated  in  the  upper  or  lower  neurone  ? 
— (Upper,  on  account  of  increased  or  normal  tendon 
reflexes,  absence  of  w^asting,  and  electrical  changes  in  the 
muscles,  with  possibly  some  active  contractures.) 

2.  Is  the  lesion  in  the  sfinal  cord  ? — Consider  the 
reactions  of  the  reflexes  (knee-jerks,  tendo-Achillis  jerks, 
ankle-clonus,  and  plantar  reflex).  If  the  lesion  is  due  to 
an  anatomical  change  in  the  lateral  columns,  these  should 
all  behave  consistently.  An  organic  lesion  of  the  lateral 
columns  which  produces  exaggeration  of  the  knee-jerks 
should,  in  the  ordinary  course  of  things,  also  give  rise 
to  an  extensor  response  of  the  plantar  reflex,  and,  in  all 


488    DISEASES   OF   THE   NERVOUS   SYSTEM 

probability,  to  an  ankle-clonus.  If,  therefore,  the  signs 
are  contradictory,  as  they  are  when  an  exaggerated  knee- 
jerk  exists  with  a  flexor  or  an  absent  plantar  response, 
suspicion  may  be  aroused  as  to  the  likelihood  of  an 
anatomical  lesion  being  present. 

On  the  other  hand,  the  absence  of  knee-jerks  entirely 
negatives  hysteria,  as  also  does  a  typical  extensor  re- 
sponse of  the  plantar  reflex. 

3.  What  is  the  condition  of  the  sphincters  ? — Retention 
of  urine  is  not  conclusive  evidence  of  either  functional 
or  organic  disease,  but  true  incontinence  is  indicative  of 
organic  change. 

4.  //  anoBsthesia  is  present,  what  is  its  distribution  ? 
— The  anaesthesia  of  organic  disease  will  be  distributed 
according  to  anatomical  rules.  In  hysteria  the  intensity 
and  distribution  of  the  anaesthesia  may  vary  from  time 
to  time,  and  usually  does  not  correspond  in  any 
way  to  the  distribution  that  would  follow  an  organic 
lesion. 

Additional  considerations.  Gait. — The  gait  in  hysteri- 
cal paralysis  assumes  a  variety  of  forms,  some  of  which 
are  obviously  too  complicated  to  depend  upon  organic 
lesions.  In  hysterical  hemiplegia  the  affected  leg  is 
dragged  along  with  the  upper  surface  of  the  toes  on 
the  ground,  instead  of  being  stiffly  swung  round  from 
the  hip- joint  as  it  is  in  the  organic  spastic  cases.  The 
hysterical  paraplegics  generally  shuffle  along  without 
being  able  to  raise  the  soles  of  the  feet  from  the  ground. 
A  further  test  devised  by  Babinski  for  distinguishing 
between  hemiplegia  of  organic  and  hysterical  origin  is 
described  under  Hemiplegia,  p.  397. 

OVER-ACTION 

Tremors. — The  intensity  of  hysterical  tremors  may 
vary  from  the  finest  to  the  coarsest  movements,  and, 
like  other  hysterical  manifestations,  they  are  apt  to 
differ  very  widely  from  time  to  time.  They  often 
closely  simulate  the  tremors  of  organic  disease,  but  can 
generally  be  distinguished  from  them  by  the  absence  of 


HYSTERIA:    MOTOR    SYMPTOMS         489 

other  symptoms  which  would  naturally  be  associated 
with  the  tremor,  and  by  the  failure  of  the  tremor  to 
behave  continuously  like  those  known  to  be  associated 
with  organic  disease. 

Spasms. — Localized  spasm  of  muscles  is  of  common 
occurrence,  and  may  be  observed  in  connection  with 
muscles  supplied  by  both  cranial  and  spinal  nerves. 
(Fig.  115.) 

Of  the  muscles  supplied  by  cranial  nerves,  the  most 
commonly  affected  are  those  of  the  eye,  and  more  rarely 
those  of  the  tongue  (Fig.  116)  and  the  jaw. 

Eyes. — Spasm  of  the  muscles  of  accommodation  is 
frequent,  as  also  are  spasmodic  ptosis  and  conver- 
gent strabismus.  The  ptosis,  which  at  first  sight  may 
appear  to  be  a  paralytic  manifestation,  is  due  to 
spasm  of  the  orbiculares  palpebrarum,  and  is  unac- 
companied by  any  compensatory  contraction  of  the 
occipito- frontalis,  such  as  occurs  with  the  paralytic 
form  of  ptosis.  In  a  few  rare  instances,  hysterical 
ptosis  due  to  relaxation  of  the  levator  palpebrse  has 
been  recorded. 

Hysterical  rigidity  and  contractures  of  the  limbs  (Fig.  117) 
are  often  exceedingly  tense,  and  any  endeavour  to  over- 
come them  by  force  only  increases  the  opposition,  nor 
does  an  attempt  to  approximate  the  points  of  insertion 
of  the  contracted  muscles  aid  their  relaxation,  as  it 
does  in  the  cases  of  organic  origin.  Relaxation  is, 
however,  usually  effected  during  sleep,  and  generally 
by  the  aid  of  an  anaesthetic. 

Contractures  are  especially  common  in  the  feet, 
knees,  and  hips,  and  may  be  accompanied  by  great 
pain. 

Catalepsy,  during  which  the  Hmbs  remain  for  a  long 
time  in  any  position  in  which  they  are  placed,  some- 
times occurs. 

Spasms  of  respiratory  muscles. — Irregular  spasmodic 
action  of  the  muscles  of  respiration  is  associated  with 
hysterical  hiccough,  yawning,  cough,  and  globus.  Hys- 
terical dyspnoea  may  also  be  present. 


490    DISEASES   OF   THE   NERVOUS   SYSTEM 

Spasms  of  the  (Esophagus  with  difficulty  in  swallowing 
may  suggest  the  presence  of  a  stricture  of  organic 
origin. 

Convulsions. — Hysterical  convulsions,  which  in  many 


Pig.  115.— Hysterical  contractui'es  in  a  child. 
{From  a  case  under  the  care  of  Mr.  T.  H. 
Kellock.) 

respects  bear  a  close  resemblance  to  epileptic  fits, 
may  occur.  They  are  usually  ushered  in  by  some 
hysterical  sensation,  as  the  globus  hystericus,  flatu- 
lence, or  palpitation.  Generalized  tonic  spasms  then 
occur,  and  the  patient  falls  or  slides  to  the  ground,  but 


HYSTERIA:    MOTOR    SYMPTOMS  491 

seldom  with  tlie  sudden  helplessness  that  is  so  charac- 
teristic of  epilepsy.  The  tonic  stage  is  succeeded  by 
clonic  movements  which  are  often  very  wild  and  un- 
controlled, and  in  severe  cases  opisthotonos  and  different 
elaborate  postures  of  the  body  are  assumed.     After  a 


Fig.  116.— Hysterical  spasm  of  the  tongue. 
(Fierre  Marie.) 

variable  time,  usually  much  longer  than  in  epilepsy,  the 
movements  cease,  and  the  normal  mental  condition 
gradually  returns.  The  main  diagnostic  points  between 
these  fits  and  epilepsy  are  mentioned  in  the  chapter  on 
Epilepsy  (p.  442). 

Lastly,  among  the  infinite  variety  of  spasms  and 
movements  that  are  met  with  may  be  mentioned  the 
dancing  movements  known  as  saltatory  chorea. 


492    DISEASES   OF   THE   NERVOUS   SYSTEM 

Sensory  Symptoms 
Anaesthesia. — ^Anaesthesia  is  the  symptom  most  con- 
stantly   present    in    hysteria.      The    variability    of    its 
distribution  and  intensity  is  well  known,  but  the  most 


Fig.  117.— Hysterical  spasm  of  the  arm.    {Pierre  Marie.) 

frequent  distribution  is  over  one  half  of  the  body,  the 
distal  portion  of  a  limb,  or  a  segment  of  a  limb  (Fi^s. 
118,  119,  120).  It  may  also  be  present  in  irregular 
patches. 

In  hemianaesthesia  the   loss  may  be  complete  for  all 
kinds  of  sensation,  or  it  may  be  partial,  or  there  may 


HYSTERIA:    SENSORY    SYMPTOMS        493 

be  loss  for  one  kind  of  sensation  and  not  for  another. 
The  sensory  loss  usually  extends  to  the  middle  line  of 
the  body  and  affects  mucous  membranes  as  well  as 
skin.  In  some  instances  the  sensations  of  the  deeper 
structures,  e.g.  muscles,  bones,  joints,  and  ligaments, 
are  lost;  and  in  such  cases,  if  the  eyes  are  closed, 
the  patient  does  not  recognize  the  position  or  even  the 
presence  of  the  limb. 

The  special  senses  are  also  often  defective  at  the  same 
time.  The  anaesthesia  may  disappear  during  sleep. 
Sometimes  a  touch  on  one  limb  is  felt  on  the  opposite 
limb  (allocheiria). 

Hyperaesthesia. — In  some  instances  sensation  is  ab- 
normally acute  over  circumscribed  areas,  and  pain  and 
tenderness  may  be  prominent  symptoms.  Pains  may 
simulate  those  of  rheumatism,  migraine,  and  angina  ; 
headaches  are  common,  and  may  be  severe  enough 
to  suggest  the  presence  of  a  tumour.  Tender  points 
(hysterogenetic  spots),  pressure  on  which  may  start 
a  convulsion,  often  exist  over  vaiious  vertebrse,  or 
below  the  mammae,  and  in  women  ever  the  ovarian 
regions. 

Visual  symptoms. — ^Allusion  has  already  been  made  to 
the  spasms  of  the  ocular  muscles.  The  fields  of  vision  are 
frequently  contracted  in  a  manner  characteristic  of  the 
disease  :  there  is  constriction  of  both  fields,  but  more  so  of 
one  than  the  other,  the  constriction  being  greatest  on  the 
side  of  the  anaesthesia.  This  condition  is  known  as  crossed 
amUyopia.  The  chart  of  the  field  of  vision  in  these  cases 
often  has  a  spiral  or  corkscrew  appearance,  owing  to  the 
field  becoming  progressively  smaller  as  it  is  being  taken. 

Hemianopia  from  hysteria,  if  it  occurs  at  all,  is  so 
rare  that  it  need  not  be  considered.  Recognition 
of  colours  is  sometimes  modified  so  that  everything 
appears  a  uniform  grey  {achromatopsia).  Short  of  this 
the  field  for  red  is  the  last  to  be  lost,  whereas  in  organic 
disease  it  usually  fails  first.  Complete  hysterical  blind- 
ness is  rare. 

Deafness^Rud  loss  of  taste  may  also  be  present  on 


494    DISEASES   OF   THE   NERVOUS   SYSTEM 

the   same   side   as  the  hemiacsesthesia.     When  loss  of 
smell  occurs  it  is  generally  bilateral. 

Gastric  symptoms. — There  may  be  loss  of  sensation 


Fig.  118.— Hysterical  hemiansesthesia. 


HYSTEKIA:    GASTRIC    SYMPTOMS        495 

of  hunger,  or  there  may  be  undue  craving  for  various 
foods.  Sometimes  there  is  vomiting.  Obstinate  con- 
stipation may  be  present. 


Fig.  119.— A  type  of  hysterical  ansesthesia. 


496    DISEASES   OF   THE   NERVOUS   SYSTEM 

Vaso-motor  disorders,  such  as  flushing,  erythematous 
eruptions,  localized  oedema,  and  even  cutaneous  haemor- 
rhages, may  occur.     Dermographism,  the  condition  in 


FiL'   120.— Types  of  liysterioal  aneesthesia. 


HYSTERIA:    DIAGNOSIS  497 

which,  wheals  are  readily  brought  out  by  drawing  the 
finger-nail  across  the  skin,  as  in  the  case  of  those  suffer- 
ing from  urticaria,  naay  also  be  present. 

Temjperature. — The  temperatures  of  extraordinary 
height  that  are  occasionally  recorded  in  hysteria  are 
probably  of  artificial  production.  A  genuine  rise  of 
temperature  over  any  length  of  time  should  arouse 
suspicions  of  the  existence  of  underlying  organic  disease. 

Mental  Symptoms 
Capriciousness,  rapid  change  of  moods,  unreasonable 
likes  and  dislikes,  are  among  the  prominent  mental 
symptoms  of  hysteria.  There  is  a  general  exaltation  or 
dej)ression  of  the  emotional  side  of  the  mind,  with 
weakening  of  the  power  of  judgment.  In  short,  the 
nervous  system  is  unstable,  and  the  most  various  re- 
actions may  result.  Cra^Hng  for  sympathy  sometimes 
exists  to  such  a  degxee  that  self-mutilation  is  practised 
in  order  to  gain  it.  In  severe  cases,  cataleptic  condi- 
tions may  be  met  with.  Amnesias,  i.e.  the  occurrence 
of  blanks  in  the  continuity  of  memory,  are  met  with,  and 
"  double  personalities  "  also  occasionally  occur. 

Diagnosis. — Hysteria  may  simulate  the  effects  of 
almost  any  organic  disease,  and  the  diagnosis  in  some 
cases  may  be  very  difficult,  for  beyond  the  decision  that 
this  or  that  symptom  is  "  functional,"  one  has  to  con- 
sider whether  there  is  a  background  of  organic  disease 
upon  which  the  hysterical  symptoms  are  superimposed. 
The  only  way  of  avoiding  mistakes  is  to  search  thoroughly 
for  evidence  of  organic  disease  in  the  past  history  and 
present  condition  of  the  patient.  The  chief  signs  of 
organic  disease  which  should  be  looked  for  are  paresis 
of  ocular  muscles,  fixed  pupils,  nystagmus,  hemianopia, 
optic  neuritis  and  atrophy,  loss  of  tendon  reflexes,  ex- 
tensor response  of  the  plantar  reflex,  a  true  ankle-clonus, 
and  incontinence  of  urine.  It  may  be  said  that  evidence 
can  be  adduced  to  show  that  some  of  these  signs  can 
appear  in  hysteria,  but  the  occurrence  of,  at  any  rate, 


498    DISEASES   OF   THE   NERVOUS   SYSTEM 

most  of  them  is  exceedingly  rare,  and  for  practical 
purposes  it  is  safe,  whenever  the  nature  of  the  case  is 
doubtful,  to  regard  them  as  indications  of  the  presence 
of  organic  disease. 

Stress  has  elsewhere  been  laid  upon  the  danger  of 
confusing  disseminated  sclerosis  with  hysteria  (p.  216), 
and  the  diagnosis  of  the  hysterical  convulsions  from 
epilepsy  has  also  been  discussed  (p.  442). 

Prognosis. — The  prognosis  is,  on  the  whole,  good, 
but  there  is  in  many  instances  a  tendency  to  relapse. 
Some  cases  recover  spontaneously,  or  respond  rapidly  to 
suitable  treatment,  but  others  may  take  months  or  even 
years  before  they  are  cured.  All  the  symptoms  may 
disappear  quite  suddenly. 

Treatment. — Mild  cases  of  hysteria  often  recover 
with  improvement  in  general  health  and  change  of 
surroundings,  especially  if  the  latter  is  accompanied  by 
effective  moral  control.  In  others  some  course  of  special 
treatment  is  indicated,  details  of  which  will  be  found  at 
p.  505. 

Among  the  drugs  found  useful  are  valerian  and 
bromides,  while  anaemia  or  general  debility  may  be 
treated  with  tonics  such  as  iron  and  arsenic. 

COMPULSION  NEUROSES 

Mental  disturbances  which  are  chiefly  characterized 
by  want  of  control  over  certain  lines  of  thought  were 
formerly  included  under  the  title  of  psychasthenia,  but 
are  now  generally  classed  as  compulsion  neuroses. 
Among  these  are  the  different  phobias,  e.g.  the  fear  of 
being  in  open  spaces  (agoraphobia)  or  in  closed  rooms 
(claustrophobia),  the  fear  of  fire  or  of  contamination,  the 
dread  of  travelling  in  trains,  and  various  other  obses- 
sions, e.g.  the  desire  to  perform  some  useless  action  such 
as  touching  consecutive  posts  of  a  railing,  or  to  repeat 
some  meaningless  words  or  sentences.  These  symptoms 
may  occur  alone,  or  may  be  associated  with  neurasthenia 
or  other  neurotic  states. 

Treatnient  should  be  conducted  along  the  same 


NEURASTHENIA  499 

physical  and  psychical  lines  as  are  described  for  neuras- 
thenia and  hysteria  (p.  505).  The  general  views  as  to 
the  origin  of  these  various  phobias  have  been  described 
at  p.  479,  and  such  methods  of  physical  and  mental 
therapy  must  be  chosen  as  seem  most  suitable  for  each 
case. 

NEURASTHENIA 

The  term  neurasthenia  has  been  largely  used  in  a 
general  sense  to  indicate  almost  any  condition  of  func- 
tional nervous  disturbance,  but  it  has  become  more 
usual  recently  to  seek  to  limit  it  to  a  particular  group  of 
symptoms  in  which  abnormal  sensations  of  fatigue  are 
the  predominant  features. 

Etiology. — What  has  been  said  of  the  etiology  of  the 
psychoneuroses  in  general  {see  p.  479  et  seq.)  apjDlies  to 
neurasthenia — that  is  to  say,  anything  which  produces 
debility  of  the  nervous  system,  whether  it  be  fatigue 
from  work  and  worry,  or  exhaustion  from  severe  illness, 
may  set  up  a  train  of  symptoms  which  causes  the 
patient  to  fix  his  attention  unduly  on  himself  and  to  feel 
symptoms  of  bodily  and  mental  fatigue  as  soon  as  he 
undertakes  the  ordinary  tasks  of  daily  life.  Painful 
repressed  memories,  sexual  conflicts  and  masturbation 
are  regarded  by  some  as  important  factors  in  the  back- 
ground. 

Symptoms. — The  symptoms  may  be  divided  into 
mental  and  bodily. 

Mental  symptoms. — Impulses  reaching  the  brain  of 
the  neurasthenic  cease  to  be  interpreted  in  the  relative 
proportions  which  they  have  previously  borne  to  one 
another.  Incidents  of  little  importance  are  magnified, 
especially  those  connected  with  health.  The  slightest 
ache  or  pain  is  often  the  starting-point  of  some  imaginary 
dreadful  disease  ;  an  unimportant  lapse  of  memory  is 
thought  to  signify  commencing  cerebral  disorder,  while  in 
the  mental  background  there  is  frequently  a  vague  in- 
describable fear  of  impending  trouble  which  entirely 
destroys  all  loeace  of  mind.     These  symptoms  may  be 


500    DISEASES   OF   THE   NERVOUS   SYSTEM 

shortly  summed  up  by  saying  that  the  patient  worries 
unnecessarily  and  loses  confidence  in  himself.  There  is 
difficulty  in  concentrating  the  mind  upon  any  intellectual 
work,  and  there  is  a  fear  of  making  mistakes  in  work, 
though  as  a  matter  of  fact  such  mistakes  are  not  gener- 
ally made.  Throughout  it  is  a  fear  of  what  the  patient 
may  do  and  of  what  may  happen  rather  than  of  what  is 
done  and  what  does  happen. 

Bodily  symptoms. — On  the  bodily  side  the  symptoms 
are  most  various. 

Pains,  crawling  sensations,  and  other  peculiarities  are 
complained  of  both  in  head  and  in  body.  The  head- 
ache is  often  characterized  by  a  sense  of  pressure  at  the 
vertex,  but  at  other  times  it  is  indistinguishable  from 
the  frontal,  vertical,  and  occipital  headaches  due  to 
other  causes.  Tenderness  and  "  weakness  "  of  the  spine 
are  frequently  complained  of.  In  the  abdomen  the 
common  accompaniments  are  indigestion,  with  which 
is  often  associated  some  dilatation  of  the  stomach, 
and  constipation.  Flatulence  is  often  a  most  trouble- 
some symptom,  especially  in  women.  Added  to  these 
are  "  sinking  "  and  ''  fainting  "  feelings.  The  heart  gener- 
ally beats  rather  faster  than  normal,  and  the  pulse-rate 
is  easily  varied  by  nervousness  or  excitement.  Sexual 
debility  may  be  present. 

The  limbs  soon  tire,  and  after  walking  there  may  be 
a  dull  pain  in  the  lumbar  region. 

Attempts  have  been  made  to  divide  up  cases  of  neuras- 
thenia, according  to  the  seat  of  the  most  prominent  symp- 
toms, into  cerebral,  spinal,  gastric,  and  cardiac  varieties, 
but  such  a  division  is  too  artificial  to  be  of  practical  use. 

Physical  signs. — On  examination,  no  signs  of  organic 
disease  will  be  discovered.  The  deep  reflexes  of  the 
arms  and  legs  may  be  normal  or  exaggerated,  but  if 
tliey  are  absent  it  is  a  sign  that  something  more  than 
neurasthenia  is  present.  The  plantar  reactions  are  of 
flexor  type,  or  in  some  cases  no  reaction  at  all  may  be 
observed.  There  is  no  ankle-clonus,  though  a  "  pseudo  -' 
clonus,  in  which  there  are  two  or  three  feebly  sustained 


NEURASTHENIA:    SYMPTOMS  501 

clonic  contractions  that  cease  when  the  foot  is  fully 
flexed,  may  be  obtained. 

The  limbs  are  feeble  and  generally  wanting  in  tone, 
but  there  is  no  definite  paralysis.  There  is  frequently 
some  fine  tremor  to  be  observed  in  the  hands. 

When  the  patient  stands  with  the  eyes  closed  there 
may  be  a  certain  amount  of  unsteadiness.  This  seems 
to  be  an  exaggeration  of  that  slight  tendency  to  sway 
which  most  people  experience  if  they  close  their  eyes 
tightly,  and  it  can  nearly  always  be  overcome  if  the 
patient  will  make  an  efiort  to  control  his  balance.  This 
swaying  in  neurasthenia,  though  classified  under  Rom- 
berg's sign,  has  nothing  in  common  with  true  mus- 
cular incoordination  such  as  is  met  with  in  tabes  and 
cerebellar  disease. 

Examination  of  the  sensory  system  does  not  reveal 
much,  except  where  cases  show  hysterical  phenomena, 
when  some  of  the  different  forms  of  anaesthesia  peculiar 
to  the  latter  may  be  found. 

As  regards  the  eyes,  the  pupils  are  generally  large, 
but  they  are  equal,  and  contract  to  light  and  during 
accommodation. 

The  movements  of  the  eyeball  are  normal,  and  there 
are  no  changes  to  be  observed  in  the  fundus.  There 
may  be  a  slight  unsteadiness  on  extreme  lateral  fixation, 
but  there  is  no  real  nystagmus.  There  is,  however, 
often  an  error  of  refraction,  to  which  allusion  will  again 
be  made  when  discussing  treatment. 

The  history  reveals  no  symptoms  that  can  be  defi- 
nitely associated  with  organic  changes. 

There  will  have  been  no  double  vision,  though 
patients  may  complain  of  mistiness  of  sight,  which 
must  be  distinguished  from  true  diplopia ;  no  loss 
of  control  over  the  sphincters  will  be  found. 

On  testing  the  higher  functions  of  the  nervous  system 
there  may  be  signs  of  weakness  of  power  of  concen- 
tration and  rapid  onset  of  fatigue.  The  results  of  the 
"  time  discrimination  "  test,  as  described  at  p.  67,  are  fre- 
quently below  the  average,  and  it  is  often  instructive  to 


502    DISEASES   OF   THE   NEEVOUS   SYSTEM 

note  the  increased  rapidity  with  which  the  test  can  be 
carried  out  as  improvement  takes  place  under  treatment. 

As  already  stated, -the  symptoms  are  largely  of  au- 
tonomic origin,  and  it  is  therefore  disturbances  of  this 
system  that  should  be  looked  for.  Indications  may 
be  found  in  the  skin  by  the  irritability  of  the  vaso- 
motor system  (dermographism)  and  the  muscles  of  the 
hair-follicles  ("goose-skin").  The  regularity  or  other- 
wise of  the  heart-beat  can  be  chronicled  by  a  sphygmo- 
graphj  or  in  special  cases  by  a  polygraph.  Attention 
should  also  be  paid  to  the  blood-pressure,  and  especially 
in  connection  with  the  rapidity  of  the  adaptability  of 
the  vaso-motor  system  in  different  positions  of  the  body. 
It  is  probably  to  a  failure  of  such  adaptability  that 
giddiness  is  often  to  be  ascribed,  just  as  it  is  in  one 
who  is  still  weak  from  the  effects  of  a  severe  illness. 
The  oculo-cardiac  reflex  and  respiratory  arrhythmia  of 
the  pulse  may  be  tested  (p.  50). 

In  traumatic  neurasthenia  the  symptoms  are  brought 
about  by  shock  to  the  nervous  system  which  may  arise 
from  direct  injury  to  nerves,  with  the  resulting  pain  and 
exhaustion,  or  by  mental  shocks  produced  through  the 
special  senses  such  as  sight  and  hearing. 

It  thus  happens  that  a  direct  injury  or  the  fright 
and  horror  of  a  situation  may  both  or  either  set  up 
traumatic  neurasthenia. 

If,  for  example,  a  person  is  injured  bodily,  consequent 
pain  and  anxiety  may  wear  down  his  power  of  nerve 
control,  while  in  another  case  a  person  may  have  become 
neurasthenic  from  the  fear  of  what  seemed  about  to 
happen  to  himself  or  from  the  horror  experienced  at  the 
sight  of  accidents  to  others,  without  having  necessarily 
suffered  any  physical  injury. 

In  some  instances  all  the  symptoms,  including  the 
more  definite  mental  ones,  arise  immediately  after  the 
shock ;  but  in  other  cases  a  general  state  of  nervous- 
ness (the  neurasthenic  state)  precedes  more  definite 
mental  symptoms,  as  indicated  in  the  table  on  p.  482. 
There  is  sometimes  in  traumatic  neurasthenia  a  latent 


NEURASTHENIA:  DIAGNOSIS  503 

period  between  tlie  time  of  the  shock  and  the  onset  of 
symptoms. 

Diagnosis. — It  will  be  seen  from  what  has  been 
written  above  that  symptoms  included  under  neuras- 
thenia merge  into  those  of  other  forms  of  neurosis  so 
that  exact  classification  may  not  be  possible.  Similar 
symptoms  are  also  in  a  general  way  apt  to  be  present 
at  the  beginning  of  many  other  diseases,  some  of  the 
principal  of  which  it  is  as  well  to  bear  in  mind  while 
examining  the  patient. 

General  paralysis  of  the  insane  may  begin  with  symp- 
toms which  closely  resemble  those  of  neurasthenia,  and 
until  some  more  definite  signs  develop  it  may  be  impos- 
sible to  be  sure  of  the  diagnosis.  As  a  rule,  however, 
the  resemblance  is  more  apparent  than  real.  The 
important  point  to  which  attention  should  be  particu- 
larly given  is  the  exact  nature  of  the  mental  change 
that  is  present,  especially  as  regards  character  and  work. 
The  general  paralytic  is  not  only  unable  to  work  so 
well,  but  he  loses  his  skill  and  also  his  finer  sentiments. 
The  neurasthenic  is  over-anxious  about  himself,  worries, 
and  is  unable  to  keep  his  attention  fixed  for  long,  but 
he  does  not  show  the  tendency  to  mental  degeneracy 
that  is  seen  in  the  general  paralytic.  He  may  be  over- 
anxious about  his  work  and  afraid  of  making  mistakes, 
but,  as  a  matter  of  fact,  he  seldom  does  make  them; 
while,  on  the  other  hand,  the  failing  mental  powers  of 
the  general  paralytic  are  often  obvious  to  everyone 
but  himself. 

Definite  failure  of  memory,  marked  alteration  of  re- 
action times,  and  other  signs  of  degeneration  as  shown 
by  mental  tests  are  important  points  to  be  considered. 
The  presence  of  any  signs  of  organic  disease,  such  as 
inequality  of  the  pupils  with  loss  of  reaction  to  light, 
at  once  makes  it  evident,  of  course,  that  something  more 
than  neurasthenia  is  present. 

Neurasthenics  are  often  depressed,  but  if  the  de- 
pression is  accompanied  by  suicidal  tendency  and 
delusions,  then  the  case  should  no  longer  be  regarded  as 


504    DISEASES   OF   THE  NERVOUS   SYSTEM 

one  of  ordinary  neurasthenia,  but  should  be  looked  upon 
as  melancholia,  so  that  proper  nursing  and  supervision 
may  be  provided  to  ensure  the  best  chances  of  recovery. 
The  presence  of  definite  anaesthesias,  amnesias,  and 
paralyses  will  help  in  the  differentiation  of  hysteria, 
but  often  no  sharp  definition  can  be  drawn.  When 
the  patient  complains  of  being  tired  after  walking  a 
little  way  it  is  well  to  bear  in  mind  the  possibility  of 
myasthenia  gravis,  which  in  its  less  pronounced  forms 
may  be  confused  with  neurasthenia.  The  importance 
of  differentiating  disseminated  sclerosis  from  functional 
disease  is  insisted  on  elsewhere  (p.  217). 

Treatment. — The  general  principles  of  treatment 
are  those  described  at  jd.  505,  with  such  modifications  as 
are  required  for  any  particular  case. 

ANXIETY  NEUROSES 

The  symptoms  of  this  form  are  mainly  characterized 
by  general  irritability,  apprehensiveness  of  impending 
ills,  dizziness,  sensations  of  collapse,  together  with  bodily 
signs  usually  associated  with  fear,  such  as  dysj^noea, 
palpitations,  vaso-motor  disturbances,  tremors  and 
digestive  troubles.  Phobias  of  various  kinds  are  often 
present.  Sleep  is  frequently  disturbed  by  frightening 
dreams. 

As  will  be  seen,  the  symptoms  closely  resemble  those 
of  other  forms  of  neurosis,  but  are  always  grouped  round 
the  main  one,  which  is  a  feeling  of  anxiety. 

The  state  is  sometimes  spoken  of  as  a  neurosis  as 
distinct  from  a  psychoneurosis  because  the  anxiety, 
though  of  course  a  psychic  symptom,  is  considered  to  be 
of  somatic  origin,  arising,  according  to  the  theories  of 
Freud,  from  inadequate  psychic  reactions  in  regard  to 
sexual  life.  On  this  hypothesis  there  is  unused  psychic 
energy  in  the  mind  of  a  "  free  floating  "  nature  which 
may  remain  as  an  indefinite  feeling  or  may  become 
attached  to  some  definite  idea.  In  addition  to  psycho- 
therapy, any  physical  symptoms,  such  as  indigestion  or 
constipation,  should  be  carefully  treated. 


PSYCHONEUROSES  505 

GENERAL  SUMMARY  AND  TREATMENT 

A  classification  of  psychoneuroses  on  the  lines  indi- 
cated above,  while  useful  for  working  purposes,  must  not 
be  interpreted  too  rigidly,  since  many  of  the  symptoms 
overlap,  and  other   groups  may  be  added   and  other 
di\dsions  made  according  to  the  views  which  difierent 
authorities  take  on   the    causation    of   these    diseases. 
Moreover,  it  is  not  desirable  to  insist  on  labelling  every 
patient  with  the  name  of  a  disease,  as  symptoms  by  no 
means  always  lend  themselves  to  our  provisional  classi- 
fications.    Further,  it  will  be  seen   from  the  table  on 
p.  482  that  the  symptoms  which  can  be  classified  are 
to  a  large  extent  offshoots  from  a  common  root,  and  it 
is  to  that  root  that  we  have  to  look  for  causation  and 
indications   for  treatment.      Rows*    has  recently  em- 
phasized this  view  when  he  suggests  that  the  primary 
result  of  excessive  emotional  reactions  is  a  disturbance 
of  the  capacity  to  perceive,  to  think,  to  feel,  to  judge 
and  to  act.     This  disturbance,  he  considers,  causes  a 
condition  of  anxiety  which,  if  untreated,  will  give  rise 
to  a  sense  of  dread  as  to  future  developments  and  lay 
the  patient  open  to  receive  all  kinds  of  suggestions  from 
without  and  within.     Further  developments  will  result 
in  the  appearance  of  secondary  symptoms,  the  nature 
of  which  will    depend   largely   on    the    circumstances 
and  general  mental  make-up  of  the  patient,  and  it  is  on 
these   secondary   symptoms   that   the   classification   of 
symptoms  and  differentiation  into  diseases  are  made. 
Treatment  of  the  psychoneuroses  consists  of 
such  psychotherapeutic  methods  as  seem  most  applicable 
to  each  case,  combined  with  such  rest  and  bodily  treat- 
ment   as    may   also    be    required.     The    main    object 
underlying  the  different  forms  of  psychotherapy  is  to 
"  explore  "  the  patient's  mind  and  to  find  out  the  origin 
of  his  nervous  symptoms,  so  enabling  the  source  of  the 
trouble  to  be  recognized  and  the  ideas  to  be  rearranged 
with  a  view  to  their  better  adaptation  to  the  events  of 

*  Morison  Lectures,  Edin.  Med.  Journ..,  1920. 


506     DISEASES  OF  THE  NERVOUS  SYSTEM 

daily  life.  In  many  cases  of  recent  and  simple  origin  a 
few  conversations  in  which  the  psychological  aspects  of 
the  symptoms  are  carefully  considered  will  be  sufficient 
to  enable  the  patient  to  recover  from  his  lack  of 
adjustment,  but  in  others  the  difficulties  are  much 
greater  and  demand  a  great  deal  of  time  and  trouble 
if  they  are  to  be  overcome. 

The  most  elementary  form  of  mental  therapeutics  is 
that  known  as   simple  suggestion,  which    is  used,   con- 
sciously or  unconsciously,  by  all  practitioners.     It  is  not 
an  easy  matter   to  define  exactly  what  is  meant   by 
suggestion,   but  broadly  speaking  it  is  the  power  which 
some  people  have  of  impressing  their  views  on  others. 
It  is  met  with  in  daily  life,  and  recognized   in   people 
whose   presence   seems  to  give  others  confidence.      In 
therapeutics  it  is  shown  in  acquiring  the  confidence  of 
I^atients,  in  making  them  feel  they  are  being  helped, 
and    in     convincing    them    that     their    troubles    are 
exaggerated  and  often  without  foundation. 

By  suggestion  it  is  frequently  possible  to  start  a 
beneficial  train  of  thought  in  a  patient's  mind  that 
will  supersede  those  that  were  troubling  him.  This 
line  of  treatment  merges  into  that  known  as  persuasion, 
which,  as  elaborated  by  Dubois,  consists  in  explaining 
to  the  patient  his  illogical  trains  of  thought  and  putting 
the  circumstances  before  him  in  a  rational  light.  In 
this  way  an  attempt  is  made  to  rearrange  the  associa- 
tion of  ideas  and  so  to  re-educate  the  mind,  but,  since 
the  success  of  the  method  depends  largely  on  the 
personality  of  the  physician,  it  is  impossible  to  separate 
it  entirely  from  suggestion. 

Hypnotism  is  a  method  by  which  the  patient  is  put 
into  a  state  in  which  he  accepts  suggestions  more  easily 
than  usual.  Wingfield*  defines  hypnosis  as  "  a  psychical 
condition  in  which  suggestions  are  not  only  much  more 
easily  accepted  but  are  also  realized  with  an  intensity 
much  greater  than  is  possible  to  the  normal  state." 
This  method  has  been  found  useful  in  curing  certain 
*  "  An  Introduction  to  the  Study  of  Hypnotism,"  2nd  ed.,  1920. 


PSYCHOANALYSIS  507 

cases  of  hysterical  anaesthesias,  contractures  and  paraly- 
ses, and  also  for  recovering  lost  memories  (amnesias), 
which  are  often  associated  with  a  high  degree  of  emo- 
tional disturbance. 

The  method  introduced  by  Freud,  known  as  psycho- 
analysis, is  an  elaborate  and  lengthy  process  of  analy- 
sing the  contents  of  the  patient's  mind  with  the  object  of 
getting  at  the  root  of  the  trouble. 

Allusion  has  already  been  made  (p.  484)  to  the 
system  of  psychology  whereby  Ereud  supposes  that, 
in  certain  circumstances,  experiences,  especially  those 
of  a  painful  nature,  may  fail  to  be  duly  associated,  and, 
being  painful  to  the  individual,  may  gradually  become 
pushed  out  of  the  conscious  memory  and  relegated  to 
the  sphere  of  the  unconscious  mind,  where  they  may 
still  remain  active  and  cause  trouble. 

The  essential  point  of  Freud's  method  is  the  attempt 
to  bring  such  submerged  experiences  to  light  and  to 
associate  them  with  conscious  experiences,  whereby 
they  assume  their  rightful  proportion  among  the  events 
of  the  individual's  life,  and,  no  longer  isolated,  cease  to 
be  a  source  of  disturbance. 

The  method  by  which  this  is  sought  to  be  accom- 
plished consists,  as  the  term  psychoanalysis  implies,  in 
analysing  the  patient's  mental  contents  until  the  root 
of  the  evil  is  discovered. 

Originally  Freud  made  use  of  hypnosis  for  this 
purpose,  but  eventually  he  discarded  it  for  the  more 
simple  method  of  "  free  association,"  which  consists 
essentially  in  persuading  the  patient  to  give  out  his 
thoughts  indiscriminately  without  self-criticism  and 
regardless  of  their  seemingly  slight  importance.  By 
this  means  the  physician  picks  out  the  salient  points 
and  gradually  bores  down  to  the  offending  source. 

As  an  additional  means  of  arriving  at  the  contents 
of  a  patient's  mind,  Freud  has  ^elaborated,  at  great 
length,  his  system  of  analysing  dreams.  According  to 
him,  a  dream  represents  the  fulfilment  of  some  desire 
which  has  been  repressed  in  waking  life.     This  essential 


508     DISEASES  OF  THE  NERVOUS  SYSTEM 

point  is  generally  so  grotesquely  clothed  that  a  suc- 
cessful analysis  is  often  a  very  complicated  matter. 
The  subject  is  too  intricate  to  be  dealt  with  here, 
and  the  reader  is  referred  to  special  works  on  the 
subject. 

A  further  and  important  method  of  probing  a 
patient's  mind  is  that  of  "  word  association,"  which 
has  been  especially  elaborated  by  Jung,  and  of  which 
some  account  has  already  been  given  (p.  70). 

Where  the  nervous  symptoms  are  due  to  general 
toxaemia,  such  as,  for  example,  might  arise  from  intes- 
tinal intoxication,  pyorrhoea,  or  chronic  alcoholism,  or 
to  a  disorder  of  metabolism  such  as  might  be  caused  by 
general,  diseases  or  modification  of  some  of  the  internal 
secretions,  it  is  clearly  a  mistake  to  treat  the  nervous 
symptoms  as  constituting  the  primary  complaint,  since 
the  only  hope  of  ultimate  success  lies  in  curing  the 
disease  on  which  the  nervous  symptoms  have  been 
grafted.  On  the  other  hand,  there  are  patients  in 
whom  the  nervous  symptoms  have  occurred  primarily 
through  acute  emotional  disturbance  or  more  chronic 
stress,  as  already  intimated.  Between  these  two  classes 
there  are  those  in  whom  the  causation  is  complicated 
by  the  occurrence  of  emotional  disturbance  in  an  already 
diseased  body;  and,  further,  nervous  disturbance  of 
primary  origin  is  always,  as  stated  above,  accompanied 
by  disturbance  of  bodily  functions,  especially  those  of 
the  alimentary  and  cardio-vascular  systems. 

When  the  cases  are  regarded  from  this  point  of 
view  the  reason  that  so  many  different  forms  of  treat- 
ment are  advocated  will  be  readily  understood,  but 
success  can  only  be  gained  by  careful  adaptation  to  the 
requirements  of  each  particular  case. 

In  cases  where  the  nervousness  is  clearly  secondary 
to  some  coexisting  disease  or  disorder  of  metabolism, 
it  is  obvious  that  the  treatment  must  be  directed 
towards  the  alleviation  or  removal  of  the  cause,  the 
nervous  symptoms  being  looked  upon  for  the  time  being 
as  a  secondary  matter  and  requiring  no  form  of  treat- 


PSYCHOTHERAPY  509 

ment  other  than  that  connected  with  the  general  treat- 
ment of  the  disease  on  which  they  depend. 

Cases  of  this  class  will  not  here  be  further  considered, 
but  attention  will  be  given  to  those  in  which  the 
symptoms  are  primarily  due  *;o  a  failure  of  the  nervous 
system,  and  in  which  palpitation,  disorders  of  the 
digestion,  and  other  bodily  symptoms  are  secondary  to 
the  nerve  disturbance  and  not  the  cause  of  it. 

Mental  treatment. — It  is  difficult  to  draw  any  distinct 
line  between  the  different  methods  of  psychotherapy, 
since  one  often  merges  into  another,  and  Bernard  Hart* 
has  well  summed  up  the  matter  when  he  states  that  all 
the  methods  depend  upon  the  use  of  three  main 
principles,  viz.  suggestion,  persuasion,  and  analysis,  the 
differences  being  due  to  the  proportion  in  which  these 
principles  are  combined  and  the  technique  by  which 
they  are  applied.  In  practice,  every  case  must  be 
considered  on  its  merits.  In  many,  a  relatively  simple 
"  exploration  "  of  the  mind  is  sufficient  to  clear  up  the 
symptoms,  while  others,  especially  when,  the  symptoms 
are  of  an  hysterical  nature,  can  be  quickly  relieved  by 
suggestion  or  hypnosis.  Yet  others  may  need  the 
longer  and  more  technical  process  known  as  psycho- 
analysis, the  success  of  which,  again,  depends  greatly  on 
the  careful  selection  of  suitable  cases  and  the  discretion 
with  which  the  treatment  is  carried  out. 

Bodily  treatment. — Among  the  most  important  of  the 
disordered  physical  processes  which  often  form  the 
groundwork  of  mental  symptoms  are  fatigue  (mental  or 
physical)  and  worry. 

Everyone  is  familiar  with  the  modification  of  mental 
activities  that  is  apt  to  occur  after  a  year's  work,  when 
power  of  concentration  becomes  diminished  and  things 
appear  out  of  perspective,  so  that  trifling  worries  often 
cause  anxiety  out  of  all  proportion  to  their  importance. 
In  slight  cases  of  this  kind,  absence  from  work  and 
change  of  scene  are  generally  sufficient  to  effect  a 
cure,  and  the  worker  returns  from  his  annual  holiday 
*  Froe.  Moij.  Soc.  Med.,   1918. 


510    DISEASES   OF   THE  NERVOUS   SYSTEM 

ready  to  face  the  responsibilities  of  another  year.  Such 
cases  show  the  comparative  ease  with  which  a  cure 
can  often  be  effected  when  the  disease  is  taken  at 
an  early  stage  of  its  development.  If,  however,  the 
symptoms  seem  at  all  severe,  it  is  often  a  satisfactory 
plan  to  combine  the  holiday  with  some  definite  form 
of  treatment,  such  as  can  be  obtained  at  one  or  other 
of  the  many  good  health-resorts  at  home  or  abroad. 
There,  while  the  patient  can  enjoy  his  surroundings, 
he  will  have  part  of  his  day's  occupation  mapped  out 
for  him  and  his  general  mental  and  bodily  health  will 
be  supervised. 

In  more  severe  cases  adequate  treatment  can  only 
be  obtained  by  a  preliminary  period  of  complete  isola- 
tion from  all  worries  and  responsibilities,  and  for  such 
patients  a  short  time  in  a  nursing  home  or  some  similar 
institution,  where  psychotherapy  can  be  combined 
with  massage  and  suitable  dieting,  is  often  necessary 
before  a  more  liberal  holiday  is  undertaken. 

In  cases  where  sleeplessness  persists  in  spite  of  the 
general  treatment,  drugs  may  have  to  be  given ;  but, 
broadly  speaking,  hypnotics  should  as  far  as  possible 
be  avoided. 

Regulation  of  the  bowels  is  important.  The  symp- 
toms of  neurasthenia  are  in  a  considerable  proportion 
of  cases  associated  with  constipation.  Indeed,  some 
writers  consider  that  neurasthenia  is  nearly  always  the 
result  of  a  toxaemia  which  arises  within  the  intestines, 
and  which,  presumably,  is  made  more  intense  by 
constipation.  In  any  case,  many  of  the  patients  are 
greatly  worried  by  this  condition  of  things,  which  has 
"  got  on  their  nerves,"  and  they  have  usually  tried  to 
correct  it  in  a  spasmodic  manner,  which  only  brings 
temporary  relief. 

The  choice  of  purgatives  is  naturally  Very  large,  but 
must  be  adapted  to  the  object  in  view,  which  is  that 
of  restoring  regular  rhythmical  actions.  Until  regu- 
larity is  restored,  enemata  given  frequently  are  often 
very  useful. 


EXERCISES  AFTER  "REST  CURE"        511 

Errors  of  refraction,  if  present,  should  be  corrected, 
since  a  continuous  strain  of  the  ocular  muscles  often 
leads  to  irritability  and  exhaustion. 

Suitable  drugs  in  the  form  of  sedatives  and  tonics, 
various  forms  of  electrical  treatment,  and  hydrotherapy 
all  have  their  uses  in  selected  cases. 

The  last  phase  in  the  course  of  treatment  is  to 
prepare  the  patient  for  the  resumption  of  occupation. 

One  of  the  most  critical  periods  in  the  course  of 
''  functional  "  nervous  affections  is  that  connected  with 
return  to  work,  and  the  longer  the  patient  has  been  away 
the  more  difficult  it  is  to  recommence.  This  statement 
is  applicable  both  to  those  who  work  with  their  minds 
and  those  who  work  with  their  bodies.  It  is  unreason- 
able to  expect  a  man  of  the  labouring  class  to  do  a 
full  day's  work  at  the  termination  of  an  ordinary 
"  rest  cure,"  during  which  he  has  taken  little  or  no 
exercise  and  has  almost  certainly  put  on  weight.  The 
usual  advice  to  begin  with  light  work  is  sound  enough 
in  itself,  but  as  a  rule  cannot  be  taken  because  there 
is  no  light  work  available  ;  and  the  fact  has  to  be 
recognized  that  it  is  increasingly  difficult  for  a  man 
to  get  employment  unless  he  can  do  his  full  share  of 
work.  Moreover,  men  of  the  labouring  class,  who  have 
been  in  the  habit  of  going  to  \vork  mechanically  day  after 
day  for  a  number  of  years,  find  it  difficult  on  their  own 
initiative  to  make  a  fresh  start  when  their  routine  has 
been  so  definitely  broken.  The  result  is  that  the  patient, 
feeling  himself  unable  to  do  his  ordinary  work,  becomes 
depressed,  concludes  that  he  is  not  cured,  and  tends  to 
degenerate  physically  and  mentally  into  a  condition 
in  which  he  is  unable  to  make  any  effort  to  help 
himself.  To  remedy  this  state  of  things,  physical  exer- 
cises should  be  prescribed  as  soon  as  the  preliminary 
treatment  has  improved  the  patient's  condition  suffi- 
ciently for  them  to  be  undertaken.  The  exercises  should 
be  given  daily  for  fixed  periods,  and  gradually  increased 
until  he  is  in  thoroughly  good  physical  condition  and  fit 
to  go  straight  away  to  his  occupation. 


512    DISEASES   OF   THE   NERVOUS   SYSTEM 

On  the  mental  side,  encouragement  should  be  given  to 
occupy  the  mind  as  soon  as  there  is  a  definite  inclination 
to  do  so.  At  first  the  patient  will  often  be  content  to 
lie  in  bed  for  a  time  and  do  nothing,  but  when  the 
mind  becomes  more  active  it  is  a  mistake  to  enforce 
complete  idleness,  and  some  light  occupation,  such  as 
reading  or  needlework,  should  be  suggested  as  soon  as 
the  patient  feels  able  to  do  it.  As  further  progress  is 
made  these  occupations  may  be  extended  and  various 
others  devised  to  suit  the  intellectuality  of  the 
individual  and  the  character  of  his  work. 

It  must  not  be  forgotten  that  the  performance 
of  physical  exercises  has  a  beneficial  effect  on  the  mind, 
not  only  on  account  of  the  sense  of  well-being  produced 
as  the  patient  gets  stronger,  but  also  directly  by  the 
performance  of  voluntary  movements,  which  assists  voli- 
tion and  strengthens  the  coordination  between  mental 
processes  and  voluntary  acts. 

Towards  the  latter  part  of  the  convalescent  stage 
recreation  and  occupation  should  be  further  extended, 
and  everything  possible  should  be  done  to  enable  the 
patient  to  readjust  himself  to  his  ordinary  sur- 
roundings. 


CHAPTER    LIII 

NEURALGIA;    HEADACHE;  MIGRAINE 

NEUEALGIA 
Etiology. — Neuralgia  is  the  term  given  to  recurrent 
nerve  pains  which  do  not  depend  upon  any  obvious 
organic  lesion,  but  it  is  often  used  in  a  more  general 
sense  to  indicate  many  other  kinds  of  nerve  pain. 

The  cases  may  on  this  basis  be  divided  up  into 
frimary  and  secondary. 

Primary  neuralgia,  with  which  is  associated  no  dis- 
cernible organic  lesion,  is  especially  associated  with 
exposure  to  cold,  debility,  toxaemias,  arterio-sclerosis, 
eye-strain,  and  irritation  of  the  peripheral  ends  of  the 
nerves,  e.g.  from  carious  teeth. 

Secondary  neuralgia  often  accompanies  inflammation, 
injuries,  and  degenerations  of  nerves.  Of  the  last,  the 
pains  of  tabes  and  those  accompanying  and  following 
herpes  zoster  are  good  examples. 

Symptoms. — Pain  is  the  most  prominent  symp- 
tom ;  it  is  generally  intermittent,  of  a  sharp,  stabbing 
nature,  and  sometimes  tends  to  recur  with  great  regu- 
larity at  certain  times  in  the  day  or  night.  The  skin 
over  the  painful  area  is  often  tender  to  the  touch,  and 
in  severe  cases  the  nutrition  of  the  hairs  suffers,  so  that 
they  turn  white  or  fall  out.  Crops  of  herpes  may  appear 
over  the  painful  area. 

It  is  very  common  to  find  "  painful  spots  "  where  the 
nerve  twigs  are  superficial  and  can  easily  be  pressed 
upon. 

2  H  513 


514    DISEASES   OF   THE   NERVOUS   SYSTEM 

Attacks  of  very  acute  pain  may  be  accompanied  by 
a  reflex  spasm  of  the  muscles  situated  near  the  painful 
part.  This  is  especially  notable  in  severe  cases  of  tri- 
geminal neuralgia,  and  constitutes  the  condition  known 
as  tic  douloureux. 

Diagnosis. — Whenever  possible,  it  is  necessary  to 
distinguish  a  primary  neuralgia  from  one  of  secondary 
origin.  Endeavour  must  therefore  be  made  to  exclude 
the  probability  of  inflammation,  pressure,  injury,  or 
degeneration  as  causes. 

A  line  cannot  always  be  drawn  sharply  between 
neuralgia  and  neuritis,  but  in  well-defined  cases  the 
intermittence  of  the  pain  and  the  absence  of  anaesthesia, 
of  muscular  wasting,  and  of  any  changes  in  the  electrical 
reactions  of  the  muscles  are  indicative  of  neuralgia. 

The  lightning  pains  that  occur  in  the  course  of 
tabes  are  apt  to  be  diagnosed  as  simple  neuralgia 
before  other  signs  become  manifest.  Gummata  and 
other  tumours  must  also  be  considered  as  possible  sources 
of  irritation. 

Prognosis. — Slight  attacks  of  neuralgia,  such  as 
are  commonly  complained  of  by  people  who  are  feeling 
"  run  down,"  usually  disappear  when  the  general  health 
improves. 

Severe  and  persistent  pain  is,  however,  often  far 
more  difficult  to  cure,  especially  when  it  sets-in  in  the 
course  of  the  trigeminal  nerve,  and  in  some  cases  of 
trigeminal  neuralgia  no  treatment  short  of  removing 
the  Gasserian  ganglion  may  suffice. 

No  hard-and-fast  rules  can  be  given,  for  the  likeli- 
hood of  recovery  in  every  case  can  only  be  gauged 
after  the  effects  of  treatment  have  been  ascertained. 

Treatment. — The  objects  aimed  at  are — (1)  im- 
provement of  general  health,  (2)  removal  of  poisons  such 
as  those  of  gout  or  lead,  (3)  removal  of  sources  of  irrita- 
tion of  the  nerve-endings,  (4)  administration  of  such 
drugs  as  are  known  to  influence  the  pain,  (5)  injection 
of  substances  into  the  nerve  tissues,  or  destruction  of 
the  nerve  by  surgical  means. 


NEUEALGIA  515 

The  methods  of  attaining  the  first  two  objects  depend 
on  the  application  of  general  principles,  which  need  not 
here  be  discussed  in  detail ;  they  include,  chiefly,  atten- 
tion to  the  digestion  and  the  general  hygiene  of  the 
patient,  and  the  correction  of  anaemia  if  present.  The 
removal  of  all  sources  of  peripheral  irritation  is  especially 
important  in  neuralgia  of  the  trigeminal  nerve,  which  is 
often  the  result  of  eye-strain  or  decayed  teeth  ;  these 
will  again  be  alluded  to  in  the  section  on  trigeminal 
neuralgia. 

The  drugs  from  which  relief  from  neuralgia  can 
often  be  obtained  are  numerous,  but  are  not  very 
dependable. 

Phenacetin,  aspirin,  antifebrin,  and  antipyrin,  with 
preparations  of  a  kindred  nature,  are  among  the  most 
popular  of  these  drugs. 

Iodides  are  useful  in  certain  cases  where  arterio- 
sclerosis and  chronic  rheumatic  processes  appear  to  be 
the  cause  of  the  pain. 

Gelsemium  sometimes  cures  the  pain  of  facial  neur- 
algia very  successfully,  but  it  is  generally  necessary 
to  give  it  in  doses  of  sufficient  strength  to  produce 
unpleasant  physiological  effects. 

Morphia  will,  of  course,  relieve  the  pain,  but  it 
should,  needless  to  say,  be  reserved  for  the  most  occa- 
sional use  only. 

Alkalis  in  large  doses,  especially  ammonium  carbonate 
and  ammonium  chloride,  are  often  useful. 

Externally,  ointments  of  menthol  and  other  sub- 
stances that  dull  the  sensibility  of  the  nerve-endings 
are  valuable  ;  and  where  there  are  any  chronic  inflam- 
matory changes  round  the  nerve,  massage  and  galvanism 
may  afiord  relief. 

In  cases  where  the  pain  does  not  yield  to  the  ordinary 
methods  of  treatment  the  question  of  obtaining  relief 
by  injections  into  the  nerve  may  be  considered.  This 
method  was  introduced  by  Schlosser,  and  has  been 
elaborated  by  Brissaud,  Sicard,  Wilfred  Harris  and 
others.     It  is  based  on  the  fact  that  injection  of  absolute 


516    DISEASES   OF   THE   NERVOUS   SYSTEM 

alcohol  into  the  substance  of  a  nerve  trunk  is  followed 
by  paralysis  ;  it  is  therefore  suitable  for  use  in  cases 
where  the  source  of  the  pain  arises  in  the  periphery  of  the 
nerves,  and  is  especially  applicable  in  carefully  selected 
cases  of  trigeminal  neuralgia. 

Trigeminal  Neuralgia 

Neuralgia  of  one  or  more  branches  of  the  5th  nerve 
is  a  very  common  complaint.  The  pain  is  generally 
at  first  confined  to  one  branch;  and  the  supra-orbital 
is  the  one  which  is  most  commonly  affected.  In  its 
slighter  forms  it  responds  readily  to  treatment,  but  in 
its  more  severe  forms  it  is  most  difficult  to  cure. 

Etiology. — Of  the  peripheral  forms  of  irritation, 
those  most  likely  to  induce  trigeminal  neuralgia  are 
eye-strain  from  errors  of  refraction,  decayed  teeth,  and 
diseases  of  the  nose,  but  it  frequently  happens  that 
no  source  of  the  trouble  can  be  traced. 

Symptoms. — The  pain,  which  recurs  in  paroxysms, 
is  often  started  by  a  movement  of  the  jaw,  as  in  talk- 
ing or  eating,  and  reflex  spasm  of  the  facial  muscles 
is  an  occasional  accompaniment  and  constitutes  the 
well-known  condition  of  tic  douloureux.  The  pain  may 
extend  to  the  neck  and  arm,  and  even  over  the  trunk, 
when  the  paroxysms  are  very  severe.  There  is  often 
hypersesthesia  over  the  area  of  pain,  and  specially  tender 
spots  where  the  nerves  emerge  from  foramina.  The 
hairs  of  the  eyebrows  may  turn  grey  or  fall  out,  as 
also  may  patches  of  hair  on  the  head. 

Diagnosis. — The  possible  existence  of  a  source  of 
peripheral  or  central  irritation  is  the  first  thing  requiring 
investigation.  The  teeth  should  be  carefully  examined, 
but  even  when  they  are  decayed  the  greatest  care  must 
be  taken  before  concluding  that  they  are  the  source 
of  the  trouble,  for  in  many  cases  a  large  number  of 
teeth  have  been  sacrificed  to  no  purpose. 

The  effect  of  application  of  heat  or  cold  to  the  in- 
dividual teeth  is  a  test  largely  relied  upon  by  dental 
surgeons.     As  already  mentioned,  it  often  happens  that 


TRIGEMINAL   NEURALGIA  517 

no  source  of  nerve  irritation  can  be  found,  and  recourse 
has  then  to  be  had  to  empirical  treatment  by  some  of 
the  drugs  referred  to  in  the  last  section. 

If  no  relief  can  be  obtained,  the  question  arises  as  to 
the  advisability  of  injecting  the  nerve,  as  mentioned  in 
the  previous  section.  If  this  measure  is  decided  upon, 
one  or  other  of  the  principal  branches  should  be  pierced 
with  a  fine  needle,  and.  a  small  quantity  of  absolute 
alcohol  (90  per  cenfc.)  injected. 

The  supra-orbital  foramen  is  used  as  a  guide  to  the 
chief  branch  in  that  vicinity  and  is  not  difficult  to  locate, 
but  considerable  experience  is  necessary  to  reach  the 
supramaxillary  branch  through  the  foramen  ovale.  If 
the  injection  is  made  into  the  perineuritic  tissues,  relief 
may  still  be  obtained,  but  it  will  be  of  shorter  duration 
than  that  derived  from  piercing  the  substance  of  the 
nerve.  When  the  nerve  has  been  accurately  pierced,  as 
shown  by  the  completeness  of  the  resulting  anaesthesia, 
there  is  usually  relief  from  pain  for  at  least  six  months, 
and  perhaps  longer.  Of  41  cases  recorded  by  Wilfred 
Harris,  38  obtained  complete  relief  for  two  to  fourteen 
months  or  more,  the  shorter  periods  representing  cases 
in  which  the  injection  was  apparently  made  into  the 
perineuritic  tissue,  as  shown  by  the  absence  of  anaesthesia. 
In  some  cases  the  relief  persisted  for  several  years. 

In  a  few  instances  the  question  of  an  operation  will 
arise.  When  the  pain  is  limited  to  the  course  of  a  single 
branch,  excision  of  a  piece  of  the  nerve  is  a  simple 
operation  which  may  be  followed  by  relief,  at  any  rate 
for  a  time  ;  but  when  the  pain  is  distributed  over  the 
area  of  two  or  all  three  of  the  roots,  a  more  radical 
operation  such  as  removal  of  the  Gasserian  ganglion  is 
the  only  procedure  likely  to  give  relief. 

Neuralgia  of  the  Brachial  Plexus 

Neuralgia  in  the  course  of   the   brachial   plexus   is 

not  very  commonly  met  with.     The  general   signs  of 

neuralgia  elsewhere  are  applicable  here,  but  particular 

care  must  be  taken,  before   making   the  diagnosis,   to 


518    DISEASES   OF   THE   NERVOUS   SYSTEM 

exclude  the  presence  of  organic  disease  of  the  cord  or 
meninges,  of  tumours  in  the  thorax,  and  of  toxaemias 
of  some  constitutional  condition  such  as  renal  disease. 

Intercostal  Neuralgia 

Neuralgia  of  the  intercostal  nerves  is  frequently  met 
with.  The  pain  which  follows  the  course  of  the  inter- 
costal nerves  is  apt  to  be  more  continuous  than  in  many- 
other  varieties,  but  is  subject  to  acute  exacerbations. 

Tender  points  are  generally  present  (1)  close  to  the 
vertebral  column  where  the  posterior  branches  come  to 
the  surface,  (2)  in  the  line  of  the  axilla  corresponding 
to  the  area  supplied  by  the  lateral  branches,  and  (3) 
where  the  anterior  cutaneous  nerves  pierce  the  muscles 
to  reach  the  surface  of  the  chest. 

Intercostal  neuralgia  often  precedes  the  appearance 
of  herpes,  but  in  these  cases  it  is  scarcely  correct  to 
speak  of  the  condition  as  neuralgia,  since  it  has  been 
shown  that  the  herpes  is  accompanied  by  haemorrhagic 
and  inflammatory  changes  in  the  posterior  root  ganglion. 
The  intercostal  pains  may  persist  for  a  long  time  after 
the  herpetic  eruption  has  subsided. 

In  making  a  diagnosis  the  principal  conditions 
from  which  intercostal  neuralgia  must  be  distinguished 
are  (1)  pains  from  irritation  or  degeneration  of  nerve 
roots,  as  may  occur  in  tabes  or  tumours  of  the  cord ; 
(2)  referred  pains  from  visceral  disease;  (3)  myalgia,  in 
which  the  pain  originates  in  the  muscles ;  (4)  pains  from 
chronic  pleurisy. 

HEADACHE 

To  ensure  any  success  in  diagnosing  and  treating 
headaches  it  is  necessary  to  take  a  wide  view  of  their 
causation,  and  not  to  attribute  them  all  to  some  one 
special  fault,  such  as  an  error  of  refraction  or  an  un- 
suitable diet. 

It  is  when  headache  is  the  main  or  only  complaint 
that  difficulty  is  most  likely  to  arise,  for  when  it  is  accom 
panied  by  other  definite  symptoms  it  is  not  generally 
difficult  to  assign  to  it  its  proper  place  in  the  disease. 


HEADACHE  :  EXAMINATION  519 

The  cliief  conditions  in  which  headache  is  likely  to 
appear  as  the  most  prominent  symptom  may  be  classified 
as  follows : 

1.  Intracranial  disease. 

2.  Disorders  of  the  urinary  system; 

3.  Disorders  of  the  cardio -vascular  system 

4.  Disorders  of  the  blood. 

5.  Migraine  and  toxaemias; 
G.  Eye-strain. 

7.  Keflex  causes. 

Routine  examination  of  all  cases  is  necessary 
if  mistakes  are  to  be  avoided,  for  neither  the  position 
nor  the  character  of  the  headache  can  be  relied  on  as 
a  guide  to  the  cause,  nor  can  a  headache  which  is 
paroxysmal  be  safely  ascribed  ofihand  to  an  evanescent 
cause. 

At  the  onset  it  is  necessary  to  make  sure  that  the 
pain  is  of  the  nature  of  "headache  "  and  not  of  neuralgia, 
since  the  causation  and  treatment  of  the  latter  are  often 
very  different. 

So  far  as  the  nervous  system  is  concerned,  unless 
the  history  specially  points  in  that  direction,  it  will 
generally  be  sufficient  in  the  first  place  to  examine  the 
optic  discs,  the  reaction  of  the  pupils,  the  ocular 
movements,  and  the  reflexes — bearing  in  mind  the  possi- 
bility of  the  existence  of  intracranial  growths,  cerebral 
syphilis,  tabes,  and  general  paralysis  of  the  insane — and 
to  follow  up  the  investigation  in  greater  detail  if  any 
suspicious  signs  are  elicited.  The  results  of  the  exami- 
nation of  the  urine  will  indicate  the  presence  or  absence 
of  disease  of  the  kidney. 

In  the  cardio-vascular  system  the  tension  of  the 
arteries  must  be  noted,  for  a  persistent  low  or  high 
blood-pressure  will  sometimes  cause  headache,  even 
though  there  be  no  obvious  disease  of  the  kidneys. 

Valvular  lesions  of  the  heart  sometimes  cause  head- 
ache, either  through  anaemia,  such  as  may  be  associated 
with  aortic  regurgitation,  or  congestion  following  dilata- 


520    DISEASES   OF   THE   NERVOUS   SYSTEM 

tion  of  the  right  ventricle.  There  is  not  often  much 
danger  of  overlooking  the  cause  in  either  of  these  con- 
ditions, but  congestion  may,  of  course,  arise  from 
conditions  other  than  those  associated  with  valvular 
disease. 

After  the  presence  of  obvious  disease  of  the  larger 
systems  has  been  excluded,  the  eyes  and  the  sinuses 
may  be  thought  of,  and  when  thinking  of  the  sinuses 
it  is  well  to  remember  that  nasal  obstruction  may  be 
a  cause. 

Errors  of  refraction  and  accommodation,  due  to 
astigmatism  and  hypermetropia,  are  among  the  com- 
monest causes  of  headache,  and  are  often  associated  with 
seemingly  typical  attacks  of  migraine.  Simple  myopia 
does  not  usually  give  rise  to  headaches. 

The  migraine  and  toxsemic  group  of  headaches  is  a 
large  one.  The  characteristics  of  migraine  are  referred 
to  in  the  next  section,  and,  under  the  hypothesis  of 
toxaemias,  diet  and  intestinal  action  have  to  be  con- 
sidered, and  must  be  regulated  according  to  the  necessities 
of  the  individual  case. 

Headaches  sometimes  appear  to  occur  reflexly, 
as  the  result  of  sensory  impulses  from  the  ears,  eyes, 
and  other  channels.  Head  has  shown  that  many  head- 
aches of  this  kind  are  "  referred  "  pains  depending  on 
diseases  of  the  various  viscera. 

As  already  stated,  the  locality  and  character  of  a 
headache  are  not  usually  reliable  guides  either  to  the 
position  or  to  the  nature  of  the  lesion.  Exceptions 
are,  of  course,  sometimes  seen,  perhaps  chiefly  in  the 
case  of  the  headaches  of  intracranial  syphilis,  where 
the  painful  area  is  apt  to  be  localized  and  tender,  in  ad- 
dition to  which  there  may  be  the  further  characteristic 
of  nocturnal  exacerbations. 

In  most  cases  of  cerebral  tumour  the  headache 
produced  by  the  general  rise  of  intracranial  pressure 
does  not  admit  of  differentiation  from  that  produced 
at  the  seat  of  the  growth,  though,  taken  in  conjunction 
with   other  symptoms,   the   signs   are   sometimes   very 


HEADACHE:  TREATMENT  521 

suggestive,  as  when,  for  instance,  a  dull  pain  occurs 
over  one  half  of  the  occipital  region  and  at  the  same 
time  there  is  reason  to  suspect  the  pressure  of  a  tumour 
in  the  cerebellum. 

Treatment. — Variations  in  the  blood  supply — 
congestion,  hypersemia,  or  ansemia — though  produced  in 
many  different  ways,  seem  to  be  at  the  bottom  of  most 
headaches,  and  it  is  by  altering  the  character  of  the 
circulation  in  some  way  or  other  that  relief  is  often 
obtained; 

It  goes  without  saying  that,  whenever  possible,  the 
cause  should  be  removed ;  but  when  no  definite  cause 
can  be  found  it  is  necessary  to  try  to  ensure  the 
healthy  working  of  all  those  organs  through  which  the 
headache  is  likely  to  arise,  and  particularly  the  eyes, 
the  alimentary  canal,  and  the  kidneys.  The  eyes  must 
be  examined,  and  vision  corrected  if  necessary.  The 
bowels  must  be  kept  open  by  suitable  purgatives,  and 
the  kidneys  must  be  washed  out  by  simple  diuretics, 
of  which  water  in  plentiful  amount  is  one  of  the  best. 
At  the  same  time  that  the  output  is  being  looked 
to,  attention  must  be  paid  to  the  intake,  and  a  plain 
diet,  containing  a  minimum  amount  of  purin  bodies, 
prescribed. 

For  empirical  treatment  aspirin,  phenacetin,  anti- 
pyrin,  and  antifebrin  are  among  the  most  useful  drugs. 
Calcium  salts  have  been  used  with  benefit  in  cases  in 
which  headaches  seem  to  be  associated  with  a  deficient 
coagulative  power  of  the  blood.  It  is  said  that  the 
subjects  who  suffer  from  headaches  of  this  kind  are 
generally  of  a  "  lymphatic  type,"  i.e.  they  have  a 
heavy,  listless  expression,  with  pale  face  and  puffy 
eyelids,  and  their  headaches  are  generally  felt  most 
in  the  mornings.  The  lactate  of  calcium,  which  is 
more  palatable  than  the  chloride,  may  be  given  in 
doses  of  10  to  20  grains  three  times  a  day.  This  drug 
is  said  to  be  also  very  useful  for  relieving  the  morning 
headaches  that  follow  the  taking  of  too  much  alcohol 
on  the  previous  night.     Among  other  drugs  that  are 


522    DISEASES   OF   THE  NERVOUS   SYSTEM 

useful  are  salicylates,  bromides,  caffeine,  and  cannabis 
indica. 

Where  there  is  persistent  high  arterial  tension,  vaso- 
dilators, such  as  nitro-glycerin  and  trinitrin,  are  some- 
times given.  They  have  only  a  transitory  effect,  as  a 
rule,  and  more  general  methods  to  relieve  the  pressure 
should  be  employed. 

Excess  of  food,  by  disturbing  the  chemical  processes 
of  digestion,  can  undoubtedly  cause  headaches.  In  the 
proteid  diets  it  is  probably  the  purins  that  are  harmful, 
but  it  has  been  shown  by  Hare  that  headaches  of  a 
similar  nature  can  also  arise  from  taking  too  much 
carbohydrate  food. 

MIGRAINE  (HEMICRANIA) 

Migraine  is  a  paroxysmal  neurosis  characterized 
by  headache  and  sickness,  which  are  often  preceded 
by  visual  symptoms. 

Etiology. — More  women  suffer  than  men,  and  there 
is  a  strong  tendency  for  the  disease  to  run  in  families. 
Occasionally  other  neuroses,  such  as  hysteria  and  epi- 
lepsy, are  prominent  in  the  relatives  of  patients,  but  in 
a  large  number  of  instances  the  patient  and  his  family 
are  quite  free  from  any  other  special  form  of  nervous 
weakness. 

In  epileptics  the  fits  sometimes  alternate  with  attacks 
of  migraine,  and  it  is  generally  supposed  that  the  patho- 
logy of  migraine  is  analogous  to  that  of  epilepsy,  inas- 
much as  both  appear  to  depend  upon  a  disturbance  of 
the  cells  of  the  cerebral  cortex  ;  but  while  it  is  reasonable 
to  assume  that  the  greater  explosions  of  epilepsy  would 
sometimes  include  the  lesser  of  migraine,  clinically  there 
is,  at  any  rate,  a  very  wide  interval  between  the  average 
patient  with  migraine  and  the  sufferer  from  epilepsy, 
and  patients  who  are  subject  to  typical  attacks  of 
migraine  fortunately  show  no  special  tendency  to  become 
epileptics,  though  it  is  undoubtedly  easy  enough  to  find 
plenty  of  instances  where  both  have  occurred  and, 
perhaps,  alternated  in  the  same  person. 


MIGRAINE  :  ETIOLOGY  523 

Gout  and  gouty  tendencies  have  been  noted  in 
association  with  migraine,  and  it  has  been  considered 
by  some  that  uric  acid  is  the  actual  cause  of  migraine, 
but  of  this  there  is  not  sufficient  proof.  In  many  people 
the  first  attacks  date  from  childhood,  and  appear  to 
become  more  frequent  towards  the  middle  decades  of 
life ;  in  others  a  few  isolated  attacks  occur  from  time 
to  time.  In  those  who  are  the  subjects  of  migraine, 
attacks  are  often  induced  by  physical  or  mental  fatigue, 
indigestion,  alcohol,  and  work  requiring  close  application 
of  the  eyes. 

Errors  of  refraction  are  notably  a  cause  of  the 
attacks. 

General  summary  of  causation.  —  It  is  necessary  to 
insist  on  a  broad  view  being  taken  of  the  causation  of 
migraine  if  success  is  to  be  obtained  in  its  treatment. 
On  the  whole  it  appears  that  some  people  are  more 
prone  than  others  to  attacks  of  migraine,  and  that,  in 
them,  various  causes,  such  as  fatigue,  errors  of  refrac- 
tion, and  dietetic  indiscretions,  are  capable  of  inducing 
an  attack,  when  they  would  be  harmless  to  other  in- 
dividuals. 

The  nature  of  the  condition  that  determines  the 
tendency  to  migraine  is  not  definitely  known,  but  it 
is  reasonable  to  suppose  that  it  is  a  poison  generated 
either  in  the  alimentary  canal  or  in  the  tissues  themselves 
during  the  chemical  processes  taking  place  in  the  latter, 
and  that  it  acts  upon  the  brain  cells  either  directly  or 
indirectly  by  the  changes  produced  in  the  cerebral 
circulation. 

Vaso -motor  spasm,  followed  later  by  dilatation,  has 
always  been  a  plausible  hypothesis,  and  is  supported  by 
the  pallor  of  the  face  and  extremities  which  is  often 
observed  at  the  beginning  of  an  attack  and  is  followed 
later  by  flushing  ;  on  the  other  hand,  experiments  have 
not  shown  the  presence  of  vaso-constrictor  nerves  in 
the  cerebral  vessels.  It  has  also  been  suggested  that 
the  attacks  may  be  due  to  periodical  enlargement  of  the 
pituitary  body. 


524    DISEASES   OF   THE   NERVOUS   SYSTEM 

Symptoms. — The  symptoms  may  be  conveniently 
divided  into  three  groups  : 

1.  Ocular. 

2.  Headache. 

3.  Sickness. 

1.  Ocular  symptoms. — In  the  majority  of  cases  the 
eye  symptoms  are  the  first  to  occur,  but  occasionally 
they  are  preceded  by  sensations  which  pass  up  a  limb, 
like,  but  of  longer  duration  than,  the  aura  of  epilepsy, 
and  are  often  accompanied  by  feelings  of  giddiness.  The 
visual  symptoms  take  various  forms,  of  which  the  chief 
are  flashes  of  light  in  front  of  the  eyes,  scotomata, 
coloured  spectra,  hemianopia,  and  transient  general 
dimness  of  vision. 

Flashes  of  bright  light  may  start  the  attack  and 
then  spread  out  gradually  into  coloured  spectra  of  zig- 
zag shape. 

The  scotoma  is  usually  nearly  in  the  middle  of  the 
field  of  vision,  and  shows  itself  to  the  patient  by  the 
existence  of  a  black  patch  in  whatever  he  looks  at, 
the  edges  of  the  patch  being  zig-zagged,  from  which 
the  condition  has  received  the  names  of  "  fortification 
spectra  "  and  "  teichopsia."  The  coloured  spectra  may 
show  themselves  as  zig-zag  ribands  of  different  colours. 

Hemianopia  is  of  the  simple  or  homonymous  variety, 
i.e.  there  is  a  corresponding  loss  of  half-vision  in  each 
eye,  so  that  the  sight  is  lost  towards  the  right  or  the 
left,  as  the  case  may  be.  This  often  comes  on  very 
quickly,  and  the  patient  finds  he  is  only  able  to  see  one 
half  of  the  objects  he  looks  at.  At  other  times  there 
is  a  general  dimness  of  vision,  and  complete  blindness 
for  a  time  has  occasionally  been  observed. 

2.  Headache.  —  Following  on  the  eye  symptoms 
comes  the  headache.  This  often  begins  at  one  spot 
in  the  region  of  the  right  or  left  temple,  and  thence 
spreads  over  one  half  (hemicrania)  or  the  whole  of  the 
head.  At  other  times  the  painful  spot  on  the  one  side 
is  followed  by  a  pain  over  the  corresponding  area  of 
the  other  side  before  the  headache  becomes  general. 


MIGRAINE:    TREATMENT  525 

3.  Sickness. — The  headache  generally  increases  in 
intensity  for  a  time,  and  is  succeeded  by  a  sensation 
of  nausea,  which  may  be  followed  by  retching  and 
vomiting ;  the  headache,  in  its  turn,  fades  away,  and 
so  the  attack  passes  off. 

Additional  sensory  symptoms  which  sometimes  occur 
are  numbness  and  tingling  or  even  temporary  weakness 
of  a  limb,  and  occasionally  a  passing  aphasia  may  be 
met  with. 

It  must,  of  course,  be  clearly  understood  that  all 
these  symptoms  are  not  present  in  every  case  of  migraine. 
In  many  the  headache,  with  perhaps  a  certain  amount 
of  nausea,  is  the  most  prominent  symptom,  and  in 
these  the  nature  of  the  disease  is  often  revealed  by  the 
periodicity  of  the  attacks. 

Duration. — The  duration  of  an  attack  varies  from 
a  few  hours  to  a  day  or  more.  Some  people  are  in- 
capacitated for  about  two  days,  but,  as  a  rule,  recovery 
takes  place  after  a  night's  rest. 

Prognosis  and  treatment. — Cases  in  which  a 
tendency  to  the  attacks  is  only  sHght  can  often  be  cured 
by  getting  rid  of  some  form  of  irritation  which  excites 
the  attacks.  It  is  in  this  way  that  many  patients  obtain 
relief  by  wearing  suitable  glasses. 

In  some  the  most  careful  correction  of  errors  of 
refraction  and  the  removal  of  any  other  obvious  source 
of  irritation  make  but  little  difference,  and  in  others 
again  no  error  of  refraction  of  any  importance  exists. 

Diet. — The  diet  should  be  light,  and,  although  there 
is  no  proof  that  uric  acid  is  the  cause  of  the  attacks, 
many  cases  certainly  seem  to  benefit  by  abstaining 
almost  entirely  from  meat. 

In  severe  cases  it  is  worth  while  to  go  farther  and 
try  what  a  strict  vegetarian  diet  will  do. 

Early  hours  should  be  kept,  fatigue  avoided,  and 
the  bowels  kept  regular. 

For  warding  off  attacks,  bromides  and  salicylates 
are  among  the  most  useful  drugs,  and  act  better  in 
combination  than  when  given  separately.     When  the 


526    DISEASES   OF   THE   NERVOUS  SYSTEM 

patients  usually  feel  the  symptoms  on  first  awaking, 
the  medicine  should  be  taken  the  last  thing  at  night. 
In  other  cases  it  may  be  given  twice  or  three  times  a 
day  for  a  time,  and  then  gradually  reduced. 

Very  little  can  be  done  to  cut  short  an  attack  beyond 
resting  and  applying  cold  to  the  head. 

Such  drugs  as  phenacetin,  antipyrin,  and  caffeine, 
if  taken  at  the  very  beginning,  sometimes  afford  relief ; 
but  once  the  symptoms  have  gained  a  fair  hold,  drugs 
seem  to  have  little  effect — probably  the  stomach  is  not 
in  a  suitable  condition  to  absorb  them. 

The  methods  of  treating  migraine  may  be  summed 
up  as  follows  : — 

During  attacks. 

1.  Rest  in  a  darkened  room. 

2.  Cold  to  head. 

3.  Phenacetin  Dg  6  (gr.  x),  aspirin  Dg  6  (gr.  x), 

antipyrin  Dg  6  (gr.  x),  or  antifebrin  Dg  2 
(gr.  iii). 

Between  attacks. 

1.  Avoidance  of  undue  fatigue  and  excitement. 

2.  Correction    of    errors    of    refraction    or    any 

other  source  of  peripheral  irritation. 

3.  Regulation    of   diet.     Digestible    food,    with 

restriction  of  meat, 

4.  Prescription  :   e.g.  potassii  bromidi  Dg   6-10 

(gr.  x-xv),  sodii  salicylatis  Dg  6-10  (gr. 
x-xv),  spiritus  chloroformi  Dl  12  (n\xx), 
aquam  ad  Ml  30  (§i) — taken  three  times  a 
day,  or  a  larger  dose  every  night. 

Ophthalmoplegic   Migraine 
This  is  a  somewhat  uncommon  condition,  in  which 
the  periodic  headaches  are  accompanied  by  temporary 
paralysis  of  the  third  nerve  with  its  attendant  symptoms 
of  ptosis,  strabismus,  and  fixed  dilated  pupil. 


CHAPTER   LIV 

VERTIGO 

Etiology. — A  sensation  of  giddiness  may  accompany  a 
great  many  morbid  conditions,  of  which  the  most  im- 
portant are  disorders  of  the  ears,  eyes,  heart,  stomach, 
and  kidneys ;  intracranial  growths,  especially  those  con- 
nected with  the  cerebellum  ;  and  epilepsy.  Alcoholism, 
excessive  smoking,  and  other  toxsemic  conditions  may 
also  crause  vertigo. 

Aural  Vehtigo 

Disorders  of  the  ear  are  one  of  the  most  common 
causes  of  giddiness. 

So  far  as  the  ear  is  concerned,  the  sense  of  equilibrium 
depends  on  the  pressure  of  the  endolymph  and  on  the 
state  of  the  nerve  terminals  in  the  semicircular  canals, 
which  through  the  vestibular  nerve  form  communica- 
tions with  the  cerebellum.  Anything,  therefore,  that 
varies  the  pressure  or  alters  the  irritability  of  the  nerves 
may  upset  the  normal  equilibrium  and  cause  vertigo. 

The  pressure  of  the  endolymph  within  the  semi- 
circular canals  may  be  modified  by  chronic  inflamma- 
tory changes  in  the  middle  ear,  which  may  be  of  gouty, 
senile,  or  syphilitic  origin,  whereby  the  intensity  of  the 
vibrations  conducted  through  the  chain  of  ossicles  is 
increased.  Wax,  which  is  one  of  the  commonest  causes 
of  vertigo,  probably  produces  its  effects  in  the  same 
way,  for  a  solid  piece,  if  pressing  against  the  drum  of  the 
ear,  serves  as  an  additional  conductor,  and  causes  the 
intensity  of  the  vibrations  reaching  the  labyrinthine 
nerves  to  be  increased.     In  other  instances  an  undue 

627 


528    DISEASES   OF   THE   NERVOUS   SYSTEM 

irritability  of  the  nerve  fibres  or  their  centres  is  the  cause 
of  the  vertigo.  The  labyrinth  itself  may  be  the  seat  of 
injuries,  of  efiusions,  or  of  inflammations. 

The  symptoms  of  aural  vertigo  are  also  sometimes 
associated  with  high  arterial  tension  and  Bright's  disease, 
quite  apart  from  the  giddiness,  which  may  be  a  symptom 
of  uraemia. 

Vertigo  of  labyrinthine  origin  is  known  as  Meniere's 
disease,  but  the  latter  term  is  often  used  to  include  other 
forms  of  aural  vertigo. 

Symptoms. — In  a  typical  case  of  aural  vertigo  the 
patient  suddenly  feels  giddy,  and  either  has  a  sensation 
of  movement  or  actually  moves  ;  at  other  times,  apparent 
movement  of  surrounding  objects  is  the  chief  thing 
noticed.  In  severe  cases  the  patient  may  suddenly  be 
thrown  to  the  ground  or  may  only  save  himself  by  holding 
on  to  some  neighbouring  support. 

After  the  giddiness  has  lasted  a  variable  time  it  is 
often  succeeded  by  nausea  and  vomiting.  Occasionally 
temporary  diplopia  and  nystagmus  have  been  noticed, 
the  latter  being  due  to  the  disturbance  of  coordination 
which  exists  between  the  movements  of  the  head  and 
eyes  {see  p.  92). 

Tinnitus  often  immediately  precedes  a  paroxysm 
and  sometimes  also  persists  between  the  attacks ;  some 
degree  of  deafness  is  frequently  to  be  found  on  one  or 
other  side.  The  nature  of  the  deafness  can  be  tested 
by  a  tuning-fork.  The  vibrations  from  a  tuning-fork 
placed  on  the  forehead  are  ordinarily  heard  in  both 
ears  equally,  but  if  the  conducting  apparatus  of  the 
ear  is  impaired  they  are  heard  loudest  on  the  side  of 
the  deaf  ear. 

On  the  other  hand,  when  the  deafness  is  of  nerve 
origin  the  vibrations  of  the  tuning-fork  placed  on  the 
forehead  are  heard  best  on  the  sound  side. 

Further,  in  nerve  deafness,  after  the  vibrations  of 
the  tuning-fork  placed  on  the  mastoid  have  been  lost 
they  can  generally  still  be  heard  when  the  instrument 
is  held  opposite  the  meatus. 


AURAL    VERTIGO  529 

Diagnosis.— Attacks  of  vertigo  associated  with  deaf- 
ness and  tinnitus  are  tlie  main  points  to  be  established. 
And  further  evidence  can  be  obtained  by  carrying  out 
the  tests  for  labjrinthine  disease  described  at  p.  92. 

The  vertigo  of  epilepsy  is  sometimes  dijB&cult  to 
differentiate,  but  if  the  attacks  are  repeated  frequently, 
some  positive  signs  of  epilepsy,  in  the  shape  of  loss  of 
consciousness,  convulsions,  biting  the  tongue,  and  in- 
voluntary micturition,  are  almost  sure  to  be  obtained. 
The  most  difficult  cases  to  decide  are  those  in  which  the 
patient  actually  falls,  for  in  those  circumstances  there  is 
naturally  some  confusion  of  mind  which  is  not  easy  to 
differentiate  from  the  loss  of  senses  due  to  epilepsy. 
If  a  history  of  loss  of  consciousness  can  be  established, 
the  condition  is  almost  certainly  one  of  epilepsy.  It  is 
doubtful  if  vertigo  of  anything  like  the  severity  described 
above  ever  arises  from  gastric  disorders,  though,  of  course, 
"  dizziness  "  and  "  giddiness  "  are  often  complained  of 
by  patients  who  suffer  from  indigestion. 

Tumours  of  the  hrain,  especially  those  of  the  cerebellum, 
are  frequently  accompanied  by  giddiness,  but  other  signs 
of  intracranial  pressure  are  generally  present. 

It  is  necessary  to  remember  that  occasionally  the 
giddiness  of  hysterical  patients  may  simulate  that  of 
Meniere's  disease,  but,  needless  to  say,  it  is  necessary 
to  be  very  cautious  before  ascribing  a  case  to  this  cause. 

Prognosis. — In  aural  vertigo  only  very  rarely 
is  such  an  acute  attack  as  Meniere  recorded  associated 
with  changes  likely  to  endanger  life.  Possibilities  of 
improvement  must,  of  course,  largely  depend  on  the 
anatomical  changes  that  have  occurred.  In  a  large 
number  of  cases  partial  relief  can  be  obtained,  and  the 
giddiness  often  gets  less  by  the  time  the  morbid  changes 
are  sufficiently  advanced  to  cause  complete  deafness. 

Treatment. — The  ear  must  be  carefully  examined, 
and  treated  if  necessary.  If  any  wax  is  present  it  must 
be  removed.  In  some  cases  this  alone  is  sufficient 
to  effect  a  cure.  If  the  giddiness  cannot  be  accounted 
for  in  this  way,  recourse  must  be  had  to  drugs,  and  of 
2  I 


530    DISEASES   OF   THE   NERVOUS   SYSTEM 

these  the  bromides,  in  15-  to  20-grain  doses  twice  or 
three  times  a  day,  are  probably  the  most  efficacious. 
Rheumatic  and  gouty  cases  may  derive  benefit  from 
salicylates  and  iodides,  which  may  be  given  in  com- 
bination with  the  bromides.  When  the  symptoms 
are  due  to  disease  of  the  middle  ear,  small  doses  of 
pilocarpine  nitrate  (jjo  to  J^  gr.)  may  be  beneficial. 
Quinine  sulphate  is  a  drug  that  was  recommended  by 
Charcot,  who  considered  it  necessary  to  give  it  in  large 
doses,  but  many  authors  believe  small  doses  to  be  equally 
efficacious.  Counter-irritation  in  the  shape  of  a  blister 
over  the  mastoid  is  sometimes  useful.  If  these  measures 
bring  no  definite  relief,  it  may  be  well  to  put  the 
patient  to  bed  for  a  week  or  two,  for  complete  rest  is 
often  a  most  useful  therapeutic  measure.  If  the  arterial 
tension  is  too  high,  purgatives  and  careful  dieting  are 
frequently  beneficial.  If  all  else  fails  and  the  attacks 
continue  to  be  unbearable,  the  question  of  obliterating 
the  semicircular  canals  may  be  entertained. 

Treatment  of  vertigo  from  other  causes. — All  forms  of 
vertigo  are  generally  relieved  to  some  extent  by  taking 
bromides,  and  any  further  course  of  treatment  must 
depend  upon  the  cause,  whether  it  be  heart,  stomach, 
arterial  degeneration,  cerebral  tumour,  or  ocular  palsy. 


CHAPTER    LV 

VASO-MOTOR    NEUROSES 

Disordered  functions  of  the  vaso-motor  nerves  are 
sometimes  the  cause  of  various  unpleasant  symptoms, 
most  of  which  can  be  grouped  under  one  of  the  follow- 
ing headings  : 

Raynaud's  disease. 
Angioneurotic  oedema. 
Erythromelalgia. 
Acroparaesthesia.' 

Raynaud's  Disease 

The  symptoms  of  Raynaud's  disease  may  be  divided 
into  three  stages  : 

{a)  Local  "  syncope,^^  in  which  the  fingers,  toes,  and 
perhaps  the  nose  and  ears,  become  pale  and  "  dead." 

(b)  Local  "  asphyxia,''^  in  which  the  initial  pallor  is 
followed  by  congestion,  so  that  the  part  becomes  blue 
in  colour. 

(c)  Gangrene,  in  which  small  parts  of  the  asphyxiated 
areas  necrose  and  separate,  leaving  little  scars. 

A  large  number  of  patients  whose  symptoms  come 
under  this  heading  never  experience  all  these  three  stages. 
Many  people  habitually  suffer  from  "  dead  "  fingers, 
especially  in  cold  weather  or  after  putting  the  hands  into 
cold  water,  and  in  some  of  these  the  stage  of  "  local  " 
asphyxia  is  also  experienced  ;  necrosis  occurs  in  com- 
paratively few. 

Etiology. — Spasms  of  the  vessels  of  the  extremities 
appear  to  be  the  cause  of  the  disease,  and  the  spasm 
is  usually  attributed  to  a  "  vaso-motor  neurosis."     What 

531 


532    DISEASES   OF   THE   NERVOUS   SYSTEM 

causes  the  neurosis  is  uncertain,  but  probably,  at  any 
rate  in  some  cases,  it  is  the  result  of  a  toxaemia.  Symp- 
toms of  a  similar  nature  to  those  described  above  may 
be  found  associated  with  syringomyelia,  and  they  are  also 
especially  prevalent  during  the  early  stages  of  some 
cases  of  rheumatoid  arthritis. 

Vaso-motor  affections  of  the  kidney  resulting  in 
paroxysmal  haemoglobinuria  are  sometimes  associated 
with  Raynaud's  disease,  and  also  closely  allied  to  it 
clinically  are  the  ordinary  chilblains  of  winter  weather. 

Friction  and  electricity  are  the  most  useful  forms  of 
treatment,  and  in  the  case  of  the  latter  the  constant 
current,  applied  daily,  is  the  most  efficacious  method  of 
administering  it.  The  hand  and  the  positive  pole  should 
be  placed  in  a  bowl  of  warm  salt  and  water,  and  the  nega- 
tive pole  should  be  applied  to  the  upper  part  of  the  limb. 

Drugs  are  of  little  direct  value,  but  those  that  have 
a  general  tonic  action  may  be  given,  and  beneficial 
results  are  sometimes  obtained  from  nitro-glycerin, 
sodium  nitrite,  and  other  vaso-dilators. 

The  symptoms  occur  in  paroxysms  and  are  always 
worse  in  cold  weather ;  hence  care  should  be  taken  to 
wear  warm  gloves  and  socks. 

Angioneurotic  (Edema 

(Edema  occurs  in  the  skin  and  sometimes  in  the 
mucous  membranes  of  different  areas  of  the  body.  At 
one  time  the  face  may  be  the  seat  of  the  swelling,  and 
at  another  the  limbs  or  the  trunk.  The  oedema  lasts 
a  variable  time  and  then  disappears.  It  gives  rise 
to  some  discomfort  and  irritation,  and  if  present  in  the 
mouth  and  pharynx  may  make  swallowing  uncomfort- 
able ;  oedema  of  the  glottis  may  occur  and  give  rise  to 
symptoms  that  are  dangerous  to  life. 

Closely  allied  to  angioneurotic  oedema  clinically  are 
the  urticarias  which  sometimes  follow  the  ingestion  of 
shell-fish,  pork,  and  some  other  foods. 

The  history  of  the  varying  amount  of  the  swelling 
generally  helps  in  distinguishing  its  nature,  but  the  urine 


ERYTHROMELALGIA  533 

should  always  be  carefully  examined  so  as  to  exclude 
the  possibility  of  Blight's  disease  being  the  cause  of  the 
oedema. 

Sometimes  more  than  one  member  of  a  family  suffers 
from  angioneurotic  oedema. 

Treatment. — It  is  not  easy  to  cure  angioneurotic 
oedema.  Attention  should  be  directed  towards  the  ali- 
mentary canal,  and  careful  dieting,  with  abstention  from 
foods  that  usually  give  rise  to  urticaria,  may  be  tried. 
With  the  same  object  in  view,  intestinal  antiseptics 
may  be  given. 

Mtro -glycerin  and  calcium  lactate  are  among  the 
drugs  that  have  been  recommended. 

It  is  important  to  maintain  a  high  standard  of  general 

health. 

Erythromelalgia 

This  condition  usually  occurs  in  the  distal  parts  of 
the  arms  or  legs.  The  arteries  dilate,  the  skin  becomes 
hot  and  red,  and  there  is  great  pain.  After  a  time  the 
redness  of  the  skin  disappears,  and  is  replaced  by  a 
bluish  colour.  The  intensity  of  the  symptoms  is  in- 
creased by  holding  the  affected  limb  in  a  dependent 
position,  and  also  by  warmth  or  exercise.  When  arte- 
rial degeneration  is  present,  intermittent  claudication 
and  cramps  may  be  associated  with  this  disease. 

Symptoms  of  erythromelalgia  may  be  met  with  in 
association  with  various  organic  diseases  of  the  nervous 
system,  e.g.  multiple  neuritis  and  tabes,  and  with  arterio- 
sclerosis ;  at  other  times  no  sign  of  organic  changes  can 
be  found,  and  the  disease  is  then  attributed  to  a  vaso- 
motor neurosis.  The  symptoms  are  generally  relieved 
by  keeping  the  limb  high  and  by  cool  applications  to  the 
affected  part.  Massage  and  electricity  may  also  be 
useful,  as  they  are  in  Raynaud's  disease. 

Acroparesthesia  of  Vaso-motor  Origin 

Here  the  patient  complains  of  tinglings,  pins  and 
needles,  and  numbness  of  the  extremities  (usually  of 
the  arms).     In  most  of  the  cases  the  symptoms  come 


534    DISEASES   OF   THE   NERVOUS   SYSTEM 

on  in  the  night.  The  patient  goes  to  bed  and  falls  asleep, 
but  after  a  time  wakes  up  with  tingling  and  numbness, 
which  may  be  sufficiently  severe  to  prevent  any  more 
sleep  being  obtained.  On  getting  up  and  using  the 
limbs  the  sensations  usually  pass  off,  but  they  are  very 
apt  to  recur  during  the  day  if  the  patient  rests  for  any 
length  of  time,  especially  if  near  a  fire  so  that  the  limbs 
become  hot. 

The  symptoms  are  more  commonly  found  in  women 
than  in  men,  and  especially  in  those  who  are  engaged 
a  good  deal  in  washing.  The  use  of  alcohol  also  seems 
to  favour  their  onset. 

The  women  sufferers  are  often  overworked  and 
generally  debilitated,  and  some  of  them  are  at  the 
menopause  period  of  life. 

Tingling,  numbness,  and  "  crawling  sensations," 
accompanied  in  some  instances  by  some  anaesthesia, 
are  frequently  met  with  over  the  area  of  the  thigh, 
which  derives  its  cutaneous  nerve  supply  from  the  ex- 
ternal cutaneous  nerve,  and  to  this  condition  the  term 
meralgia  "paresthetica  has  been  applied.  In  the  majority 
of  cases  no  lesion  of  the  nerve  can  be  found,  and 
we  have  to  fall  back  on  a  vaso-motor  neurosis  as 
the  cause. 

Diagnosis. — Before  concluding  that  the  symptoms 
of  acroparaesthesia  are  due  to  a  functional  disturbance, 
care  must  be  taken  to  exclude  the  presence  of  peri- 
pheral neuritis,  locomotor  ataxy,  or  other  organic  con- 
ditions with  which  symptoms  of  a  similar  nature  may 
be  associated. 

The  prognosis  of  these  cases  is,  on  the  whole, 
good  if  the  patient  can  be  placed  amidst  favourable 
surroundings,  but  recovery  is  generally  slow  and  the 
relapses  are  frequent. 

Treatment. — Iron,  nux  vomica,  and  other  tonics 
are  helpful,  with  a  view  to  restoring  the  health,  but  a 
thorough  change  is  best  of  all  if  it  can  be  procured. 

Electricity  and  massage  are  both  useful. 

It  is  difficult  to  prescribe  anything  which  will   be 


ACKOPARiESTHESIA  535 

certain  to  relieve  the  symptoms  at  the  time,  but  the 
bromides  seem  to  afford  as  much  relief  as  any  drug. 
If  sleep  be  much  interfered  with,  other  hypnotics  may 
be  required. 

Vaso-motor  Neuroses  of  General  Distribution 

These  neuroses  have  already  been  alluded  to  in  the 
chapter  on  the  Autonomic  System  (p.  46).  They  in- 
clude such  vaso-motor  symptoms  as  flushing,  shivering, 
palpitation,  and  dyspnoea,  many  of  which  are  asso- 
ciated with  neurasthenia  and  with  the  period  of  the 
menopause. 

The  bromides,  nux  vomica,  strychnine,  and  the  vaso- 
dilators are  among  the  drugs  that  are  likely  to  give 
relief. 


CHAPTER  LVI 

EXOPHTHALMIC    GOITRE   (GRAVES'S  DISEASE, 
BASEDOW'S    DISEASE) 

Etiology. — Exophthalmic  goitre  is  far  more  commonly 
seen  in  women  than  in  men.  In  the  majority  of  the  cases 
the  symptoms  first  appear  between  the  ages  of  15  and 
40,  and  it  is  rare  to  find  the  disease  arising  in  elderly 
people. 

In  some  instances  nothing  that  seems  to  have 
definitely  led  up  to  the  origin  of  the  disease  can  be 
traced,  but  in  a  considerable  proportion  of  the  cases 
fright,  shock,  and  long-continued  emotional  strain  of  the 
nervous  system  appear  to  bear  a  decided  relation  to  the 
onset  of  the  symptoms.  Auto-intoxication  from  the 
alimentary  canal  or  other  sources  also  appears  to  be  a 
cause. 

Pathology. — The  view  that  meets  with  most  favour 
at  the  present  day  is  that  which  attributes  the  symptoms 
to  hypersecretion  or  some  other  modification  of  the 
functions  of  the  thyroid,  and  j)ossibly  also  the  para- 
thyroid glands.  The  influence  which  the  secretion  of  the 
thyroid  gland  normally  exerts  on  the  general  metabolism 
of  the  body  can  be  fully  appreciated  from  the  ill  effects 
which  arise  on  its  removal ;  and  if  disappearance  of  the 
gland  can  produce  such  far-reaching  results,  it  is  not 
difficult  to  understand  how  modifications  in  the  com- 
l^osition  or  amount  of  the  secretion  can  produce  peculiar 
effects  of  another  nature.  This  view  receives  support 
from  the  frequency  with  which  changes  in  the  thyroid 
gland  are  found  in  exo2)hthalmic  goitre,  and  it  is  also 

536 


EXOPHTHALMIC    GOITRE:    SYMPTOMS    537 

a  noticeable  feature  that  many  of  the  symptoms  are 
almost  the  exact  opposite  of  those  which  occur  in  myx- 
oedema,  where  the  secretion  of  the  gland  has  disappeared. 
Moreover,  excessive  doses  of  th^Toid  gland  are  liable  to 
cause  symptoms  similar  to  many  of  those  associated 
with  exophthalmic  goitre ;  and,  lastly,  relief  from  the 
symptoms  of  exophthalmic  goitre  is  often  obtained  by 
ligation  of  the  arteries  or  by  partial  extirpation  of  the 
gland. 

In  some  instances  an  enlarged  thymus  gland  has  been 
found,  and  changes  of  an  inconstant  and  somewhat  in- 
definite character  have  been  noted  in  the  ganglia  of  the 
cervical  sympathetic  and  in  the  medulla. 

~  Though  the  idea  that  lesions  of  the  autonomic  system 
are  primarily  responsible  for  the  disease  has  for  the  most 
part  been  abandoned,  a  large  number  of  the  symptoms 
are  undoubtedly  produced  through  the  agency  of  this 
system,  and  the  close  relationship  that  is  known  to  exist 
between  the  internal  secretions  and  the  autonomic 
system  makes  it  apparent  how  such  a  secondary  affec- 
tion can  arise.  The  sequel  of  events,  then,  would  appear 
to  be  :  (1)  Emotional  disturbance  or  an  infection  ;  (2) 
over-activity  of  the  thyroid  gland ;  (3)  over-activity 
of  the  autonomic  system  and  general  increase  of 
metabolism. 

Symptoms. — The  onset  in  most  cases  is  gradual ; 
only  occasionally  is  it  rapid. 

The  order  in  which  the  signs  appear  differs,  and 
there  is  also  great  variation  in  their  number  and  in- 
tensity, but  the  principal  ones  are  four,  viz.  enlarge- 
ment of  the  thyroid  gland,  tachycardia,  exophthalmos, 
and  tremors. 

Enlargement  of  the  thyroid  gland. — This  sign  is  pre- 
sent at  some  period  or  another  in  very  nearly  all 
cases,  and  it  may  precede  other  symptoms  by  a  long 
time.  Like  the  other  manifestations  of  this  disease, 
the  enlargement  is  not  only  very  various  in  different 
cases,  but  is  apt  to  vary  from  time  to  time  in  the  same 
case.     In  some  patients  it  is  so  slight  as  to  be  almost 


538    DISEASES   OF   THE  NERVOUS   SYSTEM 

unnoticeable,  while  in  others  the  gland  is  symmetrically 
or  asymmetrically  enlarged  to  a  great  size.  The  vascu- 
larity of  the  enlarged  gland  is  usually  increased,  the 
pulsation  of  the  larger  arteries  running  into  it  can  be 
seen  and  felt,  and  not  infrequently  a  thrill  and  a  murmur 
over  the  tumour  can  also  be  detected.  Pressure  of  the 
gland  on  the  trachea  may  cause  dyspnoea. 

Tachycardia. — Rapidity  of  the  pulse-rate  is  a  char- 
acteristic symptom ;  from  100  to  120  beats  per  minute 
are  commonly  met  with,  and  frequently  the  number  is 
very  much  higher. 

Pulsation  of  the  heart  is  generally  more  obvious 
than  usual,  not  infrequently  there  is  some  dilatation, 
and  a  systolic  murmur  can  often  be  heard  at  the  apex. 
In  long-standing  cases  there  may  be  hypertrophy,  and 
eventually  signs  of  cardiac  degeneration  may  be  found. 

Tracings  by  means  of  the  electro-cardiograph  show 
that  the  rapidity  of  the  heart  is  due  to  an  exaggeration 
of  the  physiological  beat,  and  is  produced  at  the  expense 
of  the  diastole. 

The  blood-pressure  in  a  typical  uncomplicated  case 
is  low  owing  to  dilatation  of  the  peripheral  vessels,  the 
latter  being  one  of  the  most  frequent  features  of  the 
disease,  as  evinced  by  the  patients  generally  having  a 
moist  and  flushed  skin. 

The  interesting  observation  has  been  made  that  after 
the  thyroid  gland  has  been  operated  upon  in  Graves's 
disease  the  blood-pressure  is  very  apt  to  rise.  The 
systolic  pressure  may  even  be  doubled,  thus  throwing 
a  great  strain  on  the  heart,  and  so  in  some  cases  pos- 
sibly accounting  for  sudden  deaths  that  have  occurred 
(Strickland  Goodall). 

Exophthalmos. — This  is  not  an  invariable  symptom. 
When  it  is  present  the  prominence  of  the  eyeballs  gives 
the  striking  appearance  of  fright  to  the  face.  Protrusion 
of  one  eye  before  the  other  sometimes  occurs. 

The  cause  of  the  exophthalmos  has  been  much 
debated.  It  has  been  attributed  to  various  conditions, 
e.g.  increased  vascularity,  increase  of  fatty  tissue  at  the 


EXOPHTHALMIC   GOITRE:    SYMPTOMS   539 

back  of  the  eyeball,  and  contraction  of  Miiller's  un- 
striated  muscle,  whicli  runs  across  the  back  of  the  orbit. 

Other  ocular  symptoms  which  can  frequently  be 
observed  are  :  Von  Graefe's  sign,  in  which  the  dropping 
of  the  eyelids  is  delayed  when  the  eyes  are  directed 
downwards  ;  Stellwag^s  sign,  in  which  there  is  retraction 
of  the  upper  Ud  due  to  spasm  of  the  unstriated  muscle 
fibres  ;  Moebius's  sign,  in  which  there  is  difficulty  in 
converging  the  eyes  to  view  near  objects ;  and  Jo^roy'^s 
sign,  in  which  the  forehead  is  not  wrinkled  on  looking 
upwards  while  the  head  is  flexed  on  the  chest. 

Tremors. — ^A  fine  rhythmical  tremor  of  the  hands, 
which  can  often  be  better  felt  than  seen,  is  met  with  in 
most  of  the  cases. 

The  remainder  of  the  symptoms,  which  are  numerous, 
may  be  briefly  summarized  under  the  headings  of  the 
different  systems  to  which  they  are  attached. 

Nervous  system. — ^Patients  suffering  with  exophthalmic 
goitre  are  apt  to  be  nervous,  excitable,  and  very 
easily  upset.  They  often  sleep  badly,  and  they  may 
develop  symptoms  of  hysteria  and  other  slight  forms 
of  mental  derangement.  In  a  few  cases  the  mental 
changes  are  profound  enough  to  constitute  a  state  of 
insanity/ 

Respiratory  system. — ^In  the  more  severe  cases  attacks  of 
dyspnoea  may  occur,  apart  from  those  due  to  mechanical 
pressure  of  the  thyroid  upon  the  trachea. 

Alimentary  system. — The  appetite  is  variable,  as  also  is 
the  power  of  digestion.  Vomiting  and  diarrhoea,  more 
commonly  the  latter,  are  both  met  with. 

Renal  system. — The  urine  is  generally  plentiful  in 
amount,  and  often  contains  a  high  percentage  of  urea. 
Glycosuria  and  traces  of  albumin  are  found  in  some 
cases. 

Skin. — The  skin  is  usually  moist,  and  perspiration 
is  easily  induced.  Brown  pigmentation  is  frequently 
noticed. 

The  general  nutrition  is  poor ;  loss  of  weight  is  a  char- 
acteristic feature,  and  the  patients  are  sometimes  very 


540    DISEASES   OF   THE  NERVOUS   SYSTEM 

emaciated.      In  women  uterine  distm:bances  are  often 
met  with. 

Diagnosis. — There  is  no  ^difficulty  in  diagnosing 
a  case  in  which  the  main  symptoms  are  well  developed. 
There  is,  however,  always  a  possibility  of  overlooking  the 
nature  of  the  disease  when  general  symptoms,  such  as 
nervousness  and  loss  of  weight,  are  the  most  prominent 
features. 

Prognosis. — Improvement,  which  is  usually  a  very 
slow  process,  occurs  in  a  considerable  number  of  cases. 
In  some  the  symptoms  entirely  subside,  but  in  the 
majority  some  traces  of  exophthalmos  still  remain, 
as  also  frequently  do  the  nervous  and  excitable 
temperaments. 

A  fatal  termination  from  the  direct  effects  of  the 
disease  is  distinctly  uncommon,  but  it  occasionally 
happens  that  the  disease  runs  an  acute  course  and,  the 
patient  becoming  delirious,  death  takes  place  with  all 
the  symptoms  of  profoimd  toxaemia.  In  a  few  cases, 
where  the  functions  of  the  thyroid  gland  are  eventually 
destroyed,  the  symptoms  of  myxoedema  have  supervened. 

Treatment. — Among  the  drugs  which  are  of  most 
service,  arsenic,  belladonna,  bromides,  iron,  and  digitalis 
may  be  mentioned.  Arsenic,  chiefly  in  the  form  of  liquor 
arsenicalis,  is  one  of  the  most  popular  for  general  use  ; 
bromides  and  belladonna  are  both  serviceable  on  account 
of  their  sedative  effects.  Iron  acts  as  a  general  tonic, 
and  digitalis  is  frequently  useful  for  steadying  the 
heart.  When  the  enlargement  of  the  thyroid  is  accom- 
panied by  much  pulsation,  comfort  is  often  obtained  by 
placing  an  ice-bag  on  it. 

Applications  of  electricity  over  the  course  of  the 
cervical  sympathetic  and  over  the  thyroid  gland  some- 
times seem  to  produce  beneficial  effects.  The  inter- 
rupted and  constant  currents  may  both  be  used. 

Preparations  of  the  various  glands,  e.g.  thyroid, 
thymus,  and  suprarenal,  have  been  tried,  but  with  no 
distinctly  good  results.  One  would,  indeed,  expect 
preparations  of  thyroid  gland  to  be  harmful  if  the  hypo- 


EXOPHTHALMIC  GOITRE :  TREATMENT  541 

thesis  which  ascribes  the  disease  to  hypersecretion  be 
correct,  but,  as  a  matter  of  fact,  cases  have  been  recorded 
in  which  benefit  appears  to  have  been  obtained  from 
their  use.  Such  results  are  not  necessarily  to  be  taken 
as  invalidating  the  hypothesis  of  disordered  action  of  the 
thyroid  gland,  for  it  may  be  that  in  some  cases  the 
modification  of  the  secretion  is  one  of  quality  rather 
than  of  quantity.  A  preparation  known  as  rodagen 
(prepared  from  the  milk  of  goats  which  have  had  their 
thyroids  removed)  has  been  recommended. 

Administration  of  preparations  of  the  thymus  gland 
has  also  sometimes  appeared  to  be  followed  by  relief  of 
symptoms,  but  there  is  no  general  consensus  of  opinion 
in  favour  of  the  habitual  use  of  drugs  of  this  class. 

Such  measures  as  are  likely  to  improve  the  general 
health  should  be  recommended.  Rest,  sometimes  under 
the  Weir  Mitchell  system,  is  important,  as  also  are  quiet 
surroundings  and  general  tranquillity  of  mind. 

Application  of  X-rays  to  the  enlarged  thyroid  has 
been  tried,  and  in  some  cases  satisfactory  results  have 
been  reported. 

Surgical  treatment  is  directed  towards  reducing  the 
activity  of  the  thyroid  either  by  partial  removal  of  the 
gland  or  by  ligature  of  one  or  more  of  its  arteries.  It 
is  an  operation  of  considerable  severity  and  one  which 
by  no  means  always  results  in  a  cure,  for  which  reasons 
it  should  only  be  performed  after  due  consideration  of 
all  the  circumstances  of  each  case.  Strickland  Goodall, 
who  has  made  some  careful  observations  on  the  subject, 
considers  that  one  of  the  most  important  benefits 
obtained  by  early  operation  is  relief  of  tachycardia,  thus 
avoiding  the  danger  of  dilatation  and  permanent  cardiac 
weakness,  which  are  both  apt  to  occur  when  the  tachy- 
cardia has  continued  for  a  long  time.  Goodall  also  points 
out  that  the  rise  of  blood-pressure  which,  as  already 
stated,  is  apt  to  follow  the  operation  is  a  source  of 
danger  when  the  heart  is  debilitated,  and  necessitates 
the  patient  being  carefully  prepared  before  the  operation 
is  undertaken. 


CHAPTER    LVII 

TETANUS  (TRISMUS,  LOCKJAW) ;    HYDROPHOBIA 

TETANUS 
Etiology. — Tetanus  is  an  acute  infective  disease  resulting 
from  the  entrance  into  the  body  of  the  tetanus  bacillus. 

The  tetanus  bacillus,  first  described  by  Nicolaier,  is 
of  a  drumstick  shape  and  flourishes  chiefly  in  the  soil, 
and  from  this  it  is  in  most  cases  introduced  into  the 
body  through  punctured  or  lacerated  wounds.  Infants 
are  liable  to  be  infected  through  the  umbilical  wound 
(tetanus  neonatorum),  and  parturient  women  through 
the  placental  surface  (puerperal  tetanus). 

Pathology. — The  tetanus  bacilli  remain  in "  the 
neighbourhood  of  the  wound,  and  cause  symptoms  by 
diffusion  of  the  toxins  to  which  they  give  rise.  These 
toxins  have  a  special  affinity  for  nervous  tissue,  by 
which  they  appear  to  be  "  fixed  "  after  the  manner  of 
certain  stains. 

After  death,  minute  haemorrhages  can  be  seen  in 
different  parts  of  the  central  nervous  system,  which 
throughout  is  found  to  be  intensely  congested. 

Definite  changes  can  often  be  distinguished  in  the 
cell  bodies  of  the  brain  and  cord,  the  most  characteristic 
of  which  are  swelling  of  the  cell  bodies  and  modifica- 
tions of  the  staining  properties  of  the  chromatophile 
granules.* 

Symptoms. — After  the  patient  has  become  in- 
fected there  is  a  period  varying  in  duration  from  two  to 

*  Alexander  G.  R.  Foulcrton  and  Campbell  Thomson,  Trans.  Itoy. 
Med.  Chir.  Soc,  Ixxxiii. 

542 


TETANUS  543 

three  days  to  a  month  before  the  symptoms  appear. 
The  first  intimation  of  anything  wrong  is  generally 
a  sensation  of  stiffness  in  the  muscles  of  the  neck, 
which  is  soon  followed  by  spasms  in  the  muscles  of 
the  jaw  and  face,  by  which  the  jaw  becomes  locked 
and  the  features  distorted  to  produce  the  "  risus  sar- 
donicus."  Superadded  to  these  tonic  contractions  are 
spasms  of  the  muscles  of  the  trunk  and  limbs,  which 
occur  with  great  frequency  and  with  such  intensity  as 
to  cause  opisthotonos  and  other  contortions  of  the 
body.  The  slightest  peripheral  stimulation,  such  as 
from  a  loud  noise,  a  draught  of  air,  or  an  attempt  to 
move,  is  apt  to  bring  on  a  spasm. 

Spasms  of  the  respiratory  and  laryngeal  muscles 
give  rise  to  great  distress  and  difficulty  in  breathing, 
and  pain  produced  by  the  generalized  cramps  is  also  a 
prominent  feature. 

The  frequency  of  the  spasms  depends  upon  the 
intensity  of  the  infection.  The  temperature  may  be 
raised  slightly  or  not  at  all  during  the  earlier  stages  of 
the  disease,  but  there  may  bo  hyperpyrexia  towards  the 
end.  The  mind  usually  remains  clear,  and  death  takes 
place  from  exhaustion  and  failure  of  respiration. 

Diagnosis. — The  mode  of  onset,  characterized  by 
rigidity  of  the  muscles  of  the  neck,  jaw,  and  face,  when 
following  a  traceable  cause  of  infection,  seldom  leaves 
room  for  much  doubt  concerning  the  nature  of  the 
disease. 

Hydrophobia  is  the  disease  in  which  the  symptoms 
most  closely  resemble  those  of  tetanus,  but  in  hydro- 
phobia the  "  lockjaw  "  is  absent,  and  the  spasms  are 
especially  concentrated  on  the  muscles  connected  with 
respiration  and  swallowing,  so  that  attempts  to  drink 
are  at  once  followed  by  an  attack.  There  is  in  hydro- 
phobia also  the  likelihood  of  a  history  of  the  possibility  of 
infection  having  occurred  from  a  bite  of  a  dog  or  some 
other  animal. 

Poisoning  from  strychnine  gives  rise  to  spasms  which 
resemble  those  of  tetanus,  but  there  is  more  complete 


544    DISEASES   OF   THE  NERVOUS   SYSTEM 

flaccidity  of  the  muscles  in  the  intervals  between  the 
spasms,  and  trismus  is  absent.  Moreover,  the  onset 
is  likely  to  be  far  more  sudden. 

In  tetany  the  distribution  and  characters  of  the 
contractions  in  the  hands  and  feet  usually  obviate  any 
possibility  of  confusion,  even  when  the  spasms  invade 
the  muscles  of  the  trunk. 

Prognosis.  —  A  patient  who  is  suffering  from 
tetanus  is  always  in  a  dangerous  condition,  and  the 
mortality  is  said  to  be  more  than  80  per  cent. 

The  longer  the  incubation  period  the  better  is  the 
outlook  ;  and  when  the  spasms  have  set  in,  the  intensity 
of  the  infection  and  the  gravity  of  the  case  can  be  to 
some  extent  gauged  by  the  strength  and  frequency  with 
which  the  convulsions  occur. 

Treatment. — The  site  at  which  infection  has  taken 
place  should  be  cleansed  and  disinfected.  This  may 
influence  the  course  of  the  disease,  but,  as  already  stated, 
the  symptoms  appear  to  be  due  to  the  diffusion  of  toxins, 
and  therefore  they,  unfortunately,  do  not  necessarily 
subside  with  the  removal  of  the  bacilli. 

Treatment  by  antitoxin  gives  the  best  chance  of 
recovery,  and  should  be  carried  out  whenever  practicable. 
The  earlier  treatment  is  commenced  the  greater  are  the 
chances  of  success,  and  whenever  the^-  is  any  reason 
to  suspect  the  presence  of  tetanus  infection,  the  anti- 
toxin should  be  given  as  a  prophylactic  measure,  with 
a  view  of  neutralizing  the  virulence  of  the  toxin  before 
fixation  in  the  nerve  tissues  has  taken  place.  As  a 
prophylactic  measure  the  dose  should  be  from  1,000  to 
1,500  units  of  antitoxin.  For  curative  purposes  the 
doses  are  much  larger — 10,000  to  20,000  units  or 
more  according  to  the  severity  of  the  symptoms. 
The  antitoxin  is  best  given  by  subcutaneous  or 
intramuscular  injection ;  there  is  no  evidence  to 
show  that  intrathecal  or  intravenous  administration 
has  any  advantage. 

The  patient  should  be  isolated  in  a  darkened  room, 
and  should  as   far   as  possible  be  protected   from  all 


HYDEOPHOBIA  545 

forms  of  stimulation  whicli  are  liable  to  intensify  the 
strength  of  the  spasms. 

Drugs  must  be  used  to  produce  sedative  effects, 
and  for  this  purpose  bromides,  morphia,  cannabis  indica, 
and  chloral  will  naturally  suggest  themselves.  When 
the  convulsive  attacks  are  very  severe,  inhalation  of 
chloroform  may  be  followed  by  some  relief. 

Nutrition  must  be  maintained,  and  if  necessary  the 
patient  must  be  fed  by  a  nasal  tube. 

Periods  of  dyspnoea  may  be  tided  over  by  perform- 
ance of  artificial  respiration. 

HYDROPHOBIA  (RABIES) 

Etiology. — Hydrophobia  is  transmitted  to  man  by 
the  saliva  of  animals,  such  as  dogs,  foxes,  and  wolves, 
which  are  suffering  from  rabies. 

Pathology. — ^After  death  the  principal  alterations 
found  in  the  central  nervous  system  are  hypersemia, 
minute  haemorrhages,  and  extravasation  of  leucocytes 
into  the  perivascular  tissues. 

Symptoms. — The  incubation  period  is  very  variable , 
but  the  usual  interval  which  precedes  the  onset  of  symp- 
toms is  about  six  weeks,  though  cases  have  occasionally 
been  recorded  in  which  twelve  or  even  eighteen  months 
had  elapsed  before  the  signs  of  infection  made  their 
appearance. 

Vague  sensations  referred  to  the  seat  of  the  wound, 
which  in  the  meantime  may  have  healed  perfectly,  often 
first  lead  to  an  idea  that  something  is  wrong,  and  this 
suspicion  is  soon  confirmed  by  the  advent  of  convulsive 
attacks  somewhat  similar  to  those  of  tetanus,  but  differing 
from  them  in  the  fact  that  the  intensity  of  the  spasms  is 
especially  concentrated  on  the  muscles  concerned  in 
resj)iration  and  swallowing.  It  is  due  to  the  spasms  of 
the  latter  that  the  act  of  drinking  becomes  so  difficult, 
for  they  occur  whenever  the  patient  attempts  to  take 
fluid,  and  even  the  sight  of  water  is  often  sufficient  to 
bring  them  on. 

These  convulsive  seizures  may  spread  to  other  parts, 
2  J 


546    DISEASES   OF   THE   NERVOUS   SYSTEM 

and  are  brought  on  by  the  slightest  stimulation,  such 
as  a  sudden  noise,  or  a  draught  of  air  impinging  on  the 
body ;  when  the  muscles  of  respiration  are  implicated 
there  occur  intense  and  painful  attacks  of  dyspnoea, 
which  then  become  a  prominent  feature  of  the  case. 

The  temperature  is  raised,  and  wandering  of  the 
mind  with  hallucinations  and  delusions  is  frequently 
present. 

Death  occurs  from  exhaustion  brought  about  by  the 
spasms,  and  it  may  be  j)receded  by  a  paralysis  begin- 
ning in  the  lower  limbs,  similar  to  that  which  occurs  in 
animals. 

Diagnosis.  —  The  main  differences  between  the 
symptoms  of  hydrophobia  and  tetanus  have  already  been 
mentioned  (p.  543). 

The  convulsions  arising  from  poisoning  by  strychnine 
are  not  concentrated  in  the  muscles  concerned  in  swallow- 
ing, as  they  are  in  hydrophobia.  Tetany  can  scarcely  give 
rise  to  any  serious  difficulty,  but  hysteria  has  occasionally 
been  known  to  simulate  the  symptoms  of  hydrophobia. 

Prognosis. — The  danger  of  a  hydrophobia  occurring 
after  the  bite  of  an  animal  suffering  from  rabies  is  greater 
when  the  patient  is  bitten  on  unprotected  parts  of  the 
body,  such  as  the  face  and  hands,  than  when  the  animal's 
teeth  have  passed  through  the  clothes,  the  latter  acting 
as  a  barrier  to  the  entrance  of  the  poison.  About  15  per 
cent,  of  the  patients  bitten  by  dogs  suffering  from 
rabies  eventually  develop  symptoms  of  hydrophobia. 

Treatment. — A  recent  bite  should  be  freely  cau- 
terized as  soon  as  possible,  and,  if  none  of  the  usual 
methods  are  at  hand,  rough-and-ready  means,  such  as  the 
application  of  some  heated  instrument  to  the  wound, 
can  usually  be  devised.  Diffusion  of  the  poison  may 
be  also  retarded  by  applying  a  ligature  tightly  round  the 
limb  and  encouraging  free  bleeding. 

Once  the  poison  has  been  absorbed,  the  only  method 
of  treatment  by  which  it  can  be  hoped  to  stave  off  the 
disease  is  that  of  inoculation  with  the  attenuated  virus 
as  devised  by  Pasteur.     The  length  of  the  incubation 


HYDROPHOBIA:  TEEATMENT  547 

period  makes  it  possible  to  render  the  patient  immune 
by  the  inoculation  of  attenuated  virus  of  gradually  in- 
creasing strength,  which  is  prepared  from  the  spinal 
cords  of  rabbits  that  have  been  infected  with  the  disease. 
By  this  means  the  mortality,  which  in  untreated  cases 
is  very  high,  has  been  greatly  reduced,  and,  provided 
the  case  is  taken  in  time  and  treated  thoroughly,  there 
is  every  chance  of  the  toxin  being  neutralized  before  it 
has  had  time  to  cause  any  symptoms. 


INDEX 


Abdominal  reflex,  28 
Abducens  nerve,   anatomy  of, 
89 

paralysis  of,  89 

Abiotrophy,  239  (footnote) 
Abscess,  cerebellar,  348 

cerebral,  346 

Achromatopsia,  493 
Acromegaly,  376,  378 

symptoms      and      morbid 

anatomy  of,  378 

■ treatment  of,  384 

visual  phenomena  in,  379 

Acroparaesthesia        of        vaso- 
motor origin,    533 
Actinomycosis,      intracranial, 

351 
Adenoma^,  pituitary,  352 
Adiadochokinesis,   64,   463 
Agoraphobia,  498 
Agraphia,  414 
Alcoholic  neuritis,  153 
Alexia,  415 

Amaurotic  family  idiocy,  336 
etiology  of,  336 
pathology        of, 

337 
prognosis       and 
treatment    of, 
337 
symptoms       o  f , 
337 
Amblyopia,    crossed,    in    hys- 
teria, 493 

in   alcoholic  neuritis,   153 

toxic,  83 

Amnesia  in  hysteria,   497 
Amyotonia  congenita,   173 

diagnosis  of,   174 

•  etiology  of,  173 

prognosis  of,  175 

symptoms   of,    173 

treatment  of,  175 

Amyotrophic  lateral  sclerosis, 
234 

and    progressive 

musctilar      at- 
rophy, 223 

diagnosis  of ,  235 

etiology   of,    234 


Amyotrophic  lateral  sclerosis, 

pathology    ot, 

234 

prognosis  of,  237 

symptoms  of,  234 

treatment  of,  237 

Anaesthesia    from    lesions    of 

cord,  20 
of        peripheral 

nerves,  12 
of  posterior 

roots,  14 

in  hyst-eria,  492 

in   multiple   neuritis,    152 

Anarthria,  419 
Aneurysm,  intracranial,  352 
Angioma,  intracranial,  352 
Angioneurotic   oedema,    532 
Angular  gyrus,  functions  of,  60 

visual       centre       in, 

education  of,  412 
Anhidrosis   in    lesions   of    cer- 
vical sympathetic,  53 
Ankle-clonus,  38 
Anterior  crural  nerve,  142 

poliomyelitis,  189 

tibial  nerve,  144 

Anxiety  hysteria,  484 

neuroses,  479,  504 

Aphasia,  411 

agraphia  in,  414 

alexia  in,  415 

anarthria  in,  419 

auditory  receptive  centre 

in.  414 

doubtful   delimitation   of 

centres  in,  416 

etiology  of,  411 

examination  in,  413 

Head's    classification    of, 

419 

Marie's  views  of,  416 

motor,  413 

of  Broca,  418,  419 

of  Wernicke,   418,  419 

subcortical,  416 

transient,  in  migraine,  525 

treatment  of,  420 

word-blindness   in,   414 

word-deafness  in.  414,  416 

Apoplexy  (see  Cerebral  haemor- 
rhage) 


549 


550 


INDEX 


Apraxia,  58 

Area  lor   auditory   speech,   58 

for  hearing,  60 

for   higher   mental   f\inc- 

tions,  58 

for   language   and   recog- 

nition of  objects,  60 

for  ocular  movements,  58 

for  sensory  impulses,  60 

for  smell  and  taste,  60 

for  speech,  58 

for  vision,  60 

motor,  56 

Argyll-Robertson      pupil,      in 

general      paralysis 
of  insane,  280 

in  tabes  dorsalis,  300, 

303,  304 
Arrythmia    of    pulse,    respira- 
tory, 50,  502 
Arsenical  neuritis,  154 
Arterial  supply  of  brain,  387 
Arteries,  cerebral,  387 
Arthropathies,  247,  295 
Ascending      frontal      convolu- 
tion, 58 

neuritis,  148 

Associated  movements  in  hemi- 
plegia, 399 

Association  fibres.  54,  64 

higher,  centres  of,  55 

lower,  centres  of,  55 

Astasia-abasia,  486 

Astereognosis      (see      Stereog- 
nosis) 

Asthenic  bulbar  paralysis  (see 
Myasthenia  gravis) 

Asynergia,  463 

Ataxic  paraplegia,  237 

diagnosis  of,  238 
etiology  of,  237 
pathology  of,  237 
prognosis  of,  237 
symptoms  of,  237 
treatment  of.  238 

Ataxy,  cerebellar,  64,  335 

hereditary,  239 

Athetosis.        after        cerebral 

haemorrhage,  399 

in  cerebral  diplegia,  331 

Atrophy    (see    Trophic    symp- 
toms) 

•  optic,  83 

— —  hereditary,  84 

Auditory  centre,  412 
and   visual   centres,   com- 
munication between,  415 

nerve,  anatomy  of,  108 

lesions  of,  109 

receptive  centre.  414 

Aurae  in  epilepsy,  436 

duration  of,  437 

motor.  437 

psychical,  437 


Aurae  in  epilepsy,  sensory,  436 
Aural  vertigo,  5Z7 
Automatism,  postepileptic,  438 
Autonomic    system,    allections 
of,  53 

treatment  of,  53 

aiiatomy  of,  46 

divisions  of,  46 

functions  of,  48 

of  Langley  (see  Para- 

sympatbetic       sys- 
tem) 

reactions  of,  50 

Axial  neuritis,  84 
Axons,  i,  2 

B 

Babinski,  on  hysteria,  483 
Babinski's  sign,  29 
Basedow's  disease  (see  Exoph- 
thalmic goitre) 
Bell's  palsy,  99 
Binet-Simon  tests  for  degrees 

of  mental  deficiency,  71 
Bladder    crises   in   tabes    dor- 
salis, 300 
Brachial  plexus,  anatomy  of, 
115 

distribution  of  roots 

of,  115 
neuralgia  of,  517 
paralyses  of,  117 
Brain,  arterial  supply  of,  387 

base  of,  tumours  of,  366 

sensory  neurones  in,  22 

(see  also  Cerebellum  and 

Cerebrum,    Cortex    end 
Cortical) 
Broca's    convolution,    58,    412, 

413,  418 
Brown-Sequard   paralysis,   21 

in    spinal    tumours, 

256 
Bulb,  vascular  lesions  of,  188 
Bulbar  crises  in  postdiphthe- 
ritic neuritis,  158 
— —  paralyses.   186 

lower  neurone  type, 

187 

upper  neurone  type, 

186 
Bulbar  paralysis,  asthenic  {see 
Myasthenia  gravis) 
progressive,  188 

pseudo-,  186 

Bulbo-cavernous  reflex,  27 


Caisson  disease.  206 
Capsule,    region    of    internal, 
tumours  in.  360 


INDEX 


551 


Carcinoma,   intracranial,   351 
Cardiac  parasympathetic  sys- 
tem,  testing  of   functions 
of,  50 
Caries  of  spine,  233 
Catalepsy.  489 
Cauda  eciuina,  lesions   of,   149 

tumours  of,  257 

Caveruous    sinus,    thrombosis 

of,  408 
Centres  of  coordination,  55 

of  higher   association,   55 

of  lower  association,  55 

Cerebellar  abscess,  348 

ataxy,  64,  335 

congenital,  336 

hereditary,  241 

progressive,  336 

palsies  of  children,  335 

ataxia  in,   335 

prognosis  of,  336 

tract,  direct,  18 

tumours,  370 

Cerebellum,     connections     of, 

with  other  parts  of  ner- 
vous system,  63 

functions  and  lesions  of, 

64 

tumours  of,  370 

C'jrebral  abscess,  346 

diagnosis  of,  349 

etiology  of,  346 

pathology  of,  347 

prognosis  of,  349 

symptoms  of,  347 

treatment  of,   349 

art:ries,  387 

cortex    (see   Cortex,   cere- 
bral) 

diplegia,  330 

athetosis  in,  331 

diagnosis  of,  332 

.  prognosis  of.  333 

symptoms  of,  330 

treatment  of,  333 

embolism,  406 

diagnosis  of,  393,  407 

•  etiology  of,  406 

prognosis  of,  406 

svmptoms  of,  406 

treatment  of,  407 

hr:morrhage,  389 

artery  of,  388 

course  of  events   in, 

398 

diagnosis  of,  392 

etiology  of,  389 

hemiplegia   in,  signs 

of,  396 
into  pons  and  crus, 

400 

pathology  of.  390 

prognosis  of.  395 

symptoms  of,  390 


Cerebral  haemorrhage,  treat- 
ment of,  in  early  stage, 
394 

meningitis,  312 

pachymeningitis  externa, 

327 

palsies  of  children,  causa- 

tive   classifica- 
tion of,  329 

pathology  of,  329 

sinuses,  tnrombosis  of,  ^ud 

syphilis   (see   Syphilis) 

thrombosis,  401 

diagnosis  of,  393,  405 

etiology  of,  401 

pathology  of,  404 

prognosis  of,  404 

symptoms  of,  402 

treatment  of,  405 

tumours     (.see     Tumours, 

intracranial) 
Cerebro-spinal  fever,  319 

clinical  course  of,  322 

diagnosis  of,  322 

etiology  of,  319 

pathology  of,  320 

prognosis  of,  323 

symptoms  of,  321 

treatment  of,  323 

fluid  in  general  paralysis 

of  insane,  278 

in  meningitis,  77 

in  syphilis  of  ner- 
vous  system,  267 

in  tumours  of  cord, 

257 

meningitis,  epidemic  (see 

Cerebro-spinal  fever) 
Cerebrum,  functions  of,  54 

(see  also  Cortex  and  Cor- 

tical) 
Cervical  rib,  diagnosis  of,  119 
symptoms  of,  119 

sympathetic  system,  51 

paralysis  of,   52 

Charcot's  joint,  295 
Cheyne-Stokes     breathing     in 

cerebral  haemor- 
rhage, 391,  396 

in  tu.bercuiOus  men- 
ingitis, 315 

Chiasma,  optic,  81 

lesions  of,  84 

Cholesteatoma,  intracranial, 
352 

Chorea,  460 

diagnosis  of,  463 

electrical,  466 

etiology  of,  460 

gravis,  464 

hereditary,  466 

Huntington's,  466 

in  pregnancy,  460 

paretic,  462 


552 


INDEX 


Chorea,  pathology  of,  461 

post-hemiplegic,  399 

prognosis  of.  464 

rheumatic,  460 

saltatory,  491 

— —  senile,  466 

symptoms  of,  461 

treatment  of,  464 

Chromatolysis,  2 
Chvostek's  sign,  450 
Circumflex    nerve,    injury    to, 

12'Z 
Claudication,  intermittent,  185 
Claustrophobia,  498 
Claw-hand,  132 

in    progressive    muscular 

atrophy,  220 
Clonus,  ankle-,  38 

elbow-,  38 

jaw^-,  38 

patellar,  38 

spurious,  38 

wrist-,  38 

Cochlear  nerve,  108 

Colour  vision  in  hysteria,  493 
Coloured  spectra,  524 
Coma  from  alcohol,  392 

from  concussion,  393 

from  diabetes,  392 

from  epilepsy,  392 

from  haemorrhage,  cere- 
bral, 392 

into  pons,  400 

from  opium.  392 

from  uraemia,  392 

Combined  degeneration,  sub- 
acute, 207 

Complexes,  dissociated,  484 

Compulsion  neuroses,  498 

Conditional  reflex,  62 

Congenital  ptosis,  88 

Conjugate  deviation  in  cere- 
bral haemorrhage, 
395 

in  epilepsy,   438 

Conjunctival  reflex,  28 
Conscious  mind,  479,  480 
Contractures  and  rigidity,   39 

hysterical,   489 

Conversion   hysteria,   484 
Convolution,  precentral,  58 

third  frontal,  58 

uncinate,  60 

Convulsions.   428 

a     predisponent     to     epi- 

lepsy, 432 

diagnosis  of,  440 

in  adults,  429 

in  cerebellar  disease,  371 

in  cerebral  disease,  441 

in  childhood.  428 

in  epilepsy,  433 

Jacksonian,  357 
in  hysteria,   442,  490 


Convulsions  in  infantile  hemi- 
plegia, 334 
Coordination,  centres  of.  55 
Coprolalia,  468 
Cord  (see  Spinal  cord) 
Corpora    quadrigemina,    tum- 
ours of,  366 
Corpus  callosum,  tumours  of, 

366 
Cortex,    cerebral,    sensory    ac- 
tivity of,  23 
division  of,  into  func- 
tional areas,  55 

laminae  of,  56 

visual,  lesions  of,  86 

Cortical  localization,   55 

clinical    method    of, 

56 
developmental 
method  of,  55 

experimental  method 

of,  56 

histological     method 

of,  56 
Cranial  nerve  palsies  in  cere- 
bro-spinal 
fever,  321 
in     tabes      dor- 

salis,  302 
in     tuberculous 
meningitis,  314 
Cremasteric  reflex,  28 
Crises  in  tabes  dorsalis,  299 
Crossed     amblyopia     in     hys- 
teria, 493 

paralysis  from  cerebellar 

tumours,  372 

■  from  tumour  of  crus 

cerebri,  360,  361 

in  pontine  haemor- 
rhage, 400 

hemianaesthesia,   361 

plantar  reflex,  31 

pyramidal  tracts,   6 

Crus  cerebri,  tumours  of,  360 
Cystic    degenerations,    pituit- 
ary. 352 

Cysts,     intracranial,  352 


D 

Decompression  in  intracranial 
tumours,  374,  385 

Deep  reflexes,  26,  31 

Degeneration,    reaction  of,   43 

Deiters,  nucleus  of,  63 

Deltoid  paralysis,  122 

Dementia  paralytica  (s  a  e 
General  paralysis  of  in- 
sane) 

Dendrons,  1 

Dermographism  in  hysteria, 
496 


INDEX 


553 


Deviation,  s-econdary,  90 
jJiascJiisis,  411 
Uiphtiiexutic    neuritis,,    157 
Diplegia,  cerebral,  330 
Diplococcus        intracelkilaris. 

320.  323 
Diplopia,  90 

crossed,  90 

homonymous,  89,  90 

Direct  pyramidal  tracts,  6 
Disseminated  sclerosis,  210 

atypical  varieties  of, 

214    - 
diagnosis  of,  216 
etiology  of,  210 
pathology  of,  210 
prognosis  of,  217 

•  symptoms  of,  2jl 

treatment  of,  217 

Dissociated  complexes,  484 
Diver's  paralysis,  206 
Double  personalities,  497 
Dreams,    analysis    of,    Freud's 

system  of,  507 
Dreamy  mental  states  in  cere- 
bral tumours, 
365 

• in  epilepsy,  437 

Dyspituitarism,  378 
Dystrophia     adiposo-genitalis, 

376 
Dystrophies,  muscular,  165 


E 


Echolalia,  468 
Eighth  nerve,  108 
Electrical  chorea,  466 

•  reactions,  43 

in    myasthenia    gra 

vis,  425 

in  myotonia  congen 

ita,  473 
Electricity    and    muscle    reac- 
tions, 44 

and   nerve   reactions.    43 

therapeutic  uses  of,  44 

Eleventh    nerve,    anatomy   of, 

112,  113 
Embolism,  cerebral,  406 
Encephalitis,  acute,  341 

lethargica,  341 

diagnosis  of,  345 

morbid   anatomy  of, 

345 

prognosis  of.  344 

— — ■  symptoms  of,  342 

■  treatment  of,  346 

suppurative,  346 

Endocrinic  system.   48 

neuroses  and,  481 

Endogenous  fibres  of  posterior 

columns.  18 


Endotheliomata  of   brain,  352 

hinophthalmos  iu  lesions  of 
cervical  sympathetic,  52 

Epicritic  system,  12 

Epidemic  cerebro-spinal  men- 
ingitis (see  Cerebro- 
spinal   fever) 

eiicepiialitis   (see  Enceph- 

alitis lethargicaj 
Epigastric  reflex,  28 
Epilepsy,  432 

arrest  of  fits  in,  444 

aurse  iu,  436 

motor,  437 

-  ^ —  sensory.  436 
automatism  in,  438 
convulsions    a   predispon- 

eut  to,  432 

diagnosis  of,  440 

division  of,  into  "  essen- 
tial "  ("  idiopathic  ") 
and  "  symptomatic," 
432 

etiology  of,  432 

fit  in.  details  of,  438 

differential  diagnosis 
of,  440 

mechanism  of,  434 

in  cerebral  diplegia,  332 
Jacksonian,  357 
mania  in,  438 
minor,  439 
paralysis  in,  438 
prognosis  of,  443 
symptoms  of,  434 
treatment  of.  443 

by      elimination      of 

salt.  445 

dietetic.  444 

hygienic,  443 

■  medicinal,  445 

surgical,  448 
Epileptiform  attacks,  periodic, 

and  syphilis,  275 
Erb's   juvenile   atrophy,    169 
Erythromelalgia,  533 
Eumetria,    dependence   of,    on 

cerebellum,  64 
Exogenous   fibres   of   posterior 

columns,  17 
Exophthalmic  goitre,   536 

diagnosis  of,  540 

etiology  of,  536 

pathology  of,  536 

prognosis  of,  540 

— —  symptoms  of,  537 

■  treatment  of.  540 

■ operative,  541 

Exophthalmos,  538 
Extensor  response,  29 
Exteiyial  cutaneous  nerve,  141 

plantar  nerve,  145 

■  popliteal  nerve.  143 

rectus,  paralysis  of,  89 


55i 


INDEX 


Facial  hemiatrophy,  96 

pathology  of,  98 

symptoms  of,  96 

treatment  of,  98 

nerve,  anatomy  of,  98 

paralysis,    peripheral,    99 

— — diagnosis  of.  102 

etiology  of,  99 

late   results    of, 

105 

prognosis  of,  104 

■  symptoms  of,  101 

treatment  of,  106 

spasm,  106 

Facio  -  scapiilo  -  humeral   atro- 
phy, 168 
Family  periodic  paralysis,  473 

etiology  of,  473 

prognosis  of,  474 

symptoms  of,  473 

■  treatment  of,  474 

Fatigue  indications,  67 
Field  of  vision,  erroneous  pro- 
jection of,  91 

in  disseminated 

sclerosis,  214 
Fifth  nerve,  anatomy  of,  94 

lesions  of.  95 

trophic  s  y  m  p  - 

toms  of,  95 

neuralgia  of,  516 

Flechsig's   method   of  cortical 

localization,  55 
Flexor  response,  30 
Foot-drop,  145 
Foreconscious  mind,  480 
Fortification   spectra,   524 
Fourth    nerve,    anatomy    and 

lesions  of,  88 
Franz's    method    of    time   dis- 
crimination, 68 
"  Free    association,"    Freud's, 

507 
Frenkel's    exercises    in    tabes 

dorsalis,  310 
Friedreich's  disease.  239 

■  diagnosis  of,  241 

— —  etiology  of,  239 

pathology  of,  239 

prognosis  of.  243 

symptoms  of,  239 

■ treatment  of.  244 

• variations    of,    from 

type,   242 
Freud  on  psychoneuroses,  484, 

504 
Freud's  psycho-analysis,   507 
Frohlich's   disease,   376 
Froin's  syndrome,  78,  257 
Frontal    lobes,    functions    of 

58 
tumours  of,  363 


Gait,  "  high-stepping,"  145 

in  cerebellar  disease,  370 

in  hemiplegia,  398 

— ■—  in  hysteria,  488 

in  paraiysis  agitans,  456 

spastic,  2.i0 

Ganglionic  neuromata,  162 

Gastric  crises,  300 

General    paralysis    of    insane, 
278 

diagnosis  of,  281 

etiology    of,  278 
juvenile,  279 
pathology  of,  279 
prognosis  of,  282 
spirochaetes      in 

brain  in,  278 
symptoms  of,  280 
syphilitic     ante- 
cedents of, 278 
treatment  of ,  282 

Genito-crural  nerve^  141 

Giantism,  377 

Glioma  of  brain,  352 

of  spinal  cord,  254 

Glosso-pharyngeal  nerve,  ana- 
tomy of,  109 

lesions  of,  110 

Gluteal  muscles,  action  of,  142 

nerve,  inferior,  142 

• superior.  142 

-  reflex,  28 

Glycosuria     in     exophthalmic 

goitre,  539 
Goitre,  exophthalmic,  536 
Grand  mal  (see  Epilepsy) 
Graves's  disease,  536 
Gummata  of  brain,  351 

of  spinal  cord,  255,  273 


H 


Habit  spasms,  467 
H8emato,genous     infection     of 

central     nervous     system, 

72 
Haematomyelia,   252 

diagnosis  of,  253 

etiology  of,  252 

pathology  of,  253 

prognosis  of.  253 

symptoms  of.  252 

treatment  of,  253 

Haemorrhage  into  spinal  cord, 

252 

meningeal,  of  cord.  264 

Headache.  518 

— —  causes  of,  519 

examination  in.  519 

treatment  of,  521 

—  (see  aT.'^o  Migraine) 


INDEX 


555 


Hearing,  area  for,  60 
Heine-Medin  disease,  342 
Hemiansesthesia,  crossed,  361 

in  cerebral  liseinorrhage, 

398 

in  hysteria,  492 

in  tumours  of  crus  cere 

bri,  360 

of    internal    capsule 

360 

• of  pons,  361 

Hemianopia,  double  nasal,  85 

temporal,  85 

from  tumour  encroach- 
ing on  occipital  lobe, 
364 

— —  homonymous,  85.  364 

in  cerebral  haemorrhage, 

398 

in    tiimour3    of    internal 

capsule,  360 
Hemiatrophy,  facial,  96 
Hemichorea,  461 
Hemicrania  (see  Migraine; 
Hemiplegia,  causes  of,  401 

from      cerebral      hsemor 

rhage,  393,  396 
contractures  in, 

398 
movements      in. 

399 
pain  in.  400 
symptoms  of,  396 
mental,   398 
motor,   396 

reflex.  397 

sensory,  398 

from    tumours    of    motor 

area,  357 

infantile,  333 

treatment  of,  400 

Hereditary  ataxy,  239 

cerebellar,  335 

chorea,  466 

optic  atrophy.  84 

spastic  paraplegia,  243 

Herpes   in   intercostal   neural- 
gia, 518 

Higher    fiinctions    of    nervous 

system,  examination  of,  66 
"  High-stepping  "  gait,  145 
Hunger,  abnormal,  in  cerebral 

tumour,  366 
Huntington's  chorea.  466 
Hydatid     disease     of      spinal 

cord,  255 
"  Hydrocephalic    cry  "    in    tu- 

berculoTis  meningitis,  315 
Hydrocephalus,  338 

acute,  338 

chronic,  338 

external,  338 

internal,  338 

-^ prognosis  of,  340 


Hydrocephalus,   treatment   of, 

340 
Hydromyelia,    246 
Hydrophobia,  545 

diagnosis  of.  546 

etiology  of,  545 

pathology  of,  545 

prognosis  of,  546 

symntoms  of,  545 

treatment  of,  546 

Hyperaesthesia  in  hysteria,  493 
Hyperpituitarism,  378 
Hypnotics     in     treatment     of 

psychoneuroses,  510 
Hypnotism,  506 

Hypoglossal    nerve,     anatomy 
of    113 

■  lesions   of.    113 

Hypopituitarism,  376,  378 
Hypotonus    in   tabes   dorsalie, 
288 

lesions  producing,  64 

Hysteria,  482 

anaesthesia  in,   492 

anxiety,  484 

Babinski  on,  483 

•  catalepsy  in,  489 

■  conversion,   484 

convulsions   in,   489 

diagnosis  of,  497 

•  etiology  of,  484 

gastric  disorders  in,  494 

■  hemianaesthesia  in,  492 

hyperaesthesia  in,  493 

Janet  on,  483 

larynx  in,   487 

ocular  muscles  in.  489 

oesophagus   in,   490 

paralysis  in,  485 

prognosis    of,    498 

reflexes   in.   486 

respiratory     muscles     in, 

489 

rigidity  and  contractures 

in,  489 

spasms  in,  489 

special  senses  in,  493 

sphincters  in,  487,  488 

symptoms    of,    classifica- 
tion of,  485 

mental,  497 

motor,  485 

over-action,    488 

under   -   action, 

485 

■ sensory,  492 

temperature   in,   497 

treatment     of.     498     (see 

also        Psychoneuroses, 

treatment  of) 

tremors  in,   488 

vaso-motor    disorders    in, 

496 
: visual    disorders   in,   493 


556 


INDEX 


I 


Ilio-liypogastric   nerve,   141 
Ilio-inguinal   ueive,   141 
Impulses,    path    of,    in    cord, 

19 
Infantile  hemiplegia,  333 

diagnosis  of,  334 

symptoms  of,   334 

treatment  of.   335 

paralysis,  189 

Infection    of    central    nervous 
system,     haematogenous 
and  lymphogenous,  72 
Inferior  gluteal  nerve,   142 

oblique,  88 

rectus,  paralysis  of,   88 

Insular  sclerosis  (see  Dissemi- 
nated sclerosis) 

Intercostal  neuralgia,   518 

Intermittent  claudication,  185 

Internal   secretions,   and   neu 
roses,  481 

Interossei    muscles,    paralysis 
of,  131 

Interstitial    syphilis    (sec    Sy- 
philis, interstitial) 

Intracranial     tvimours      (see 
Tumours,  intracranial) 

Ischeemic  paralysis.   136 


Jactsonian    epilepsy,   357 

Janet's  theory  of  hysteria, 
483 

Jaw- jerk,  31 

"  Jaw-winkers,"   88 

Jendrassik's  method  of  rein- 
forcing knee-.ierk,   32 

Joffroy's   sign,  539 

Jung's  word-association  tests. 
70,  508 


Kernig's  sign,  316 
Klumpke's  paralysis.   118 
Knee-jerk,   32 
——  in  sciatica,  147 
increase  of,  33 

loss  of,  32,  33 

171  complete   division 

of  the  cord,  35 

in      postdiphtheritic 

neuritis,  157 

return  of,  after  complete 

division     of     the     cord, 
35 

flignificance  of,  32 

sustained,  463 

Korsakow'fl  pBychosis,  154 


Labyrinthine   vertigo,   527 
Landouzy  -  Dejerine     atrophy, 

168 
Landry's  paralysis,   198 

-^-  diagnosis  of,  199 

etiology  of,  198 

pathology  of,  198 

prognosis  of,  200 

symptoms  of.  198 

treatment  of.  200 

variations  of,  199 

Laryngeal  crises,  300 

•  paralysis,  110 

•  in  hysteria,  487 

spasm,  112 

Laryngismus  stridulus,   112 
Latah,  468 

Lateral  sclerosis  (see  Spastic 
paraplegia) 

amyotrophic,    234 

sinus,  thrombosis  of,  408 

Lateropulsion,   457 

Latissimus  dorsi,  paralysis  of, 
127 

Lead  neuritis,   155 

Leber's  disease,  84 

Lenticular  degeneration,  pro- 
gressive, 386 

Lenticulo-striate  arteries,  rup- 
ture of,  387,  388 

Leptomeningitis,   261,   312 

Lipoma,   intracranial,   352 

of  spinal  cord,  255 

Little's    disease,    332 
Lobes,  occipital,   60 

prefrontal,  58 

-  superior       and      middle 

frontal,  58 

temporal,  60 

Lockjaw,    542 

Locomotor    ataxy    {$ee    Tabes 

dorsalis) 
Long      subscapular     nerve, 
paralysis  of,  127 

thoracic  nerve,   124 

Longitudinal  sinus,  throm- 
bosis of,   410 

Lower  limb,  paralyses  of,  140 

plexus  paralysis,  118 

Lumbar    plexus,    distribution 

of,  140 
puncture       i  n       cerebro- 
spinal  fever,   323 

in   encephalitis   letli- 

argica,  346 

in     posterior     basic 

meningitis,  325 
in   tuberculous  men- 
ingitis, 319 
technique  of,  76 
value  of,  in   diagno- 
sis, 7 


INDEX 


557 


Ijumbar    puncture,    value    of, 

in  therapeutice,  78 
I.ymphocytosis   in  meningitis, 
77 

in     syphilis,     interstitial, 

78,  267 

parenchymatous,    78, 

267 
I.ymphogenoufi     infection      of 
central  nervous 
system,   72 

■ in  tabes 

d  o  r  - 
sails, 
286 
M 

Main-en-griffe   in    ulnar   para- 
lysis, 132 

in    progressive    muscular 

atrophy,  220 
Mania,   postepileptic,   438 
Marie,      Pierre,      and     speech 

'•  centres,"  416 
Masked  spina   bifida,   251 
Mass  reflex,  35 
Median  nerve,   134 

paralysis  of,  135 

Medical      Research      Council, 

and    injuries    of    nervoue 

system,  122 
Medulla  (see  Bulb  nnd  Bulbar) 
Memory   tests,    69 
Meniere's  disease,  528 
Meningeal      haemorrhage      of 
cord,  264 

diagnosis  of,  264 

etiology    of,    264 

prognosis  of,  264 

symptoms  of,  264 

treatment      o  f, 

265 
Meninges  and  cortex,  injuries 
to,  327 

injuries  to,  327 

Meningitis,   312 
cerebral,    312 

-  epidemic     cerebro  -  spinal 

(see       Cerebro  -  spinal 
fever) 

posterior  basic,   323 

diagnosis  of,  324 
etiology  of,  323 
pathology      o  f , 

324 
prognosis  of,  325 

symptoms  of,  324 

— ■ treatment       o  f , 

325 
serous,  325 
spinal,  261 
suppurative,  326 
tuberculous,  312 
diagnosis  of,  317 


Meningitis,     tuberculous,    eti- 
ology of,  312 

- — '- general     course     of, 

317 

prognosis  of,  317 

symptoms  of,  313 

— -  treatment  of,   319 

Meningocele,  251 

sacralis   anterior,   251 

Meningomyelitis,  271 
Meningomyelocele,  251 
Meralgia  paraesthetica,  534 
Migraine,  522 

duration    of     attack    of, 

525 

etiology  of,  522 

ophthalmoplegic,    526 

prognosis  of,  525 

symptoms  of,  524 

—  headache,   524 

ocular,   524 
sickness,  525 

treatment  ofj.   525 

Minor  epilepsy,  459 
Moebius's  sign,  539 
Monoplegia     in     tumours     of 

motor  cortex,  357 
Morvan's  disease,  248 
Motor  aphasia,  413 
■ area  of  cerebrum,  58 

neurones    [see    Neurones, 

motor) 
Multiple    neuritis     (see    Neu- 
ritis, multiple) 
Multiple  -  system      lesions      of 

cord,  180 
Muscle  sense,  14 

■  substance,  structure  and 

function  of.  42 
Muscles    of    upper    extremity, 
motor  root  supply  of,   116 
Mtiscular    atrophy,    peroneal, 
176 

diagnosis  of,  178 

etiology  of,  176 
pathology  of,  176 
symptoms  of,  176 

treatment  of,  178 

progressive,  219 

■ •  dystrophies,  165 

Musculo-cutaneous     nerve     of 
arm,  128 

of  leg,  145 

Musculo-spiral     nerve,    coux'se 

and  paralysis  of,  128 
Myasthenia    gravis,   421 

■  diagnosis  of,  425 

electrical     reactions 

in,  425 
etiology    and    patho- 
logy of,  421 
■ pro,gnosis  of,  427 

■ reflexes   in,  425 

symptoms  of,  422 


558 


INDEX 


Myasthenia  gravis,  treatment 

of,  427 
Myatonia  congenita,  173 
Myelitis,    201 

acute  ascending,  203 

syphilitic.  273 

cervical,  203 

— — -  diagnosis  of,  204 

disseminated  acute,  204 

etiology  of,  201 

pathology  of,  201 

prognosis  of,  205 

symptoms  of,  -201 

transverse  dorsal,  201 

lumbar,  203 

■  treatment  of,   205 

Myelocele.  251 
Myoclonus,  471 

diagnosis  of,  471 

etiology  of,  471 

prognosis  of,  472 

symptoms  of,  471 

treatment  of,  472 

Myopathies,  165 

classification  of,  165 

•  diagnosis  of,  170 

Erb's    juvenile    form    of, 

169 

facio  -  scapulo  -  humeral 

type  of,  168 

morbid  anatomy  of.   165 

prognosis  of,  172 

pseudo-hypertrophic,    166 

treatment  of.  172 

Myotonia  congenita,  472 

electrical      reactions 

in,  473 

■ — -  etiology  of,  47?. 

pathology   of,   472 

prognosis  and  treat- 
ment of,  473 
symptoms  of,   472 

N 

Nasal     smile     in     myasthenia 

gravis.  424 
Nerve    cell,    achromatic    sub- 
stance of,  2 

■ chromatic  substance 

of,  1 

, definition  of,  1 

of  Bell,  124 

roots,  posterior,  destina- 
tion of  fibres 
of.   15 

—  sensory       n  e  u  - 

rones  in,  14 
Nervous   system,  examination 
of     higher     functions     of, 
66 
Neuralgia.  513 

bracbial.  517 

-- —  diagnosis  of,  514 


Neuralgia,  etiology  of,  513 
intercostal,  518 

prognosis  of,  514 

symptoms  of,  513 

treatment  of,  514 

-  -  trigeminal,  516 

diagnosis  of,  516 

etiology  of,  516 

•  symptoms   of,    516 

treatment  of,  517 

Neurasthenia,  499 
— -  diagnosis   of,  503 

etiology  of,  499 

rest  cure  and,  511 

signs  of,  500 

symptoms  of,  bodily,   500 

mental.    499 

traumatic,  502 

treatment  of  {see  Psycho- 
neuroses,  treatment  of) 
Neuritis,     alcoholic,     etiologv 
of,  153 

—  pathology  of,   154 

symptoms     of,     men- 
tal, 153 

motor,  153 

sensory,  153 

arsenical,  etiology  of,  154 

symptoms   of,   154 

ascending,  148 

axial,  84 

lead,  etiology  of,  155 

symptoms   of,    155 

mtiltiple,  150 

— —  diagnosis  of,  159 

etiology  of,   150 

pathology   of.   151 

— — •  prognosis  of,  160 

— ■ —  - — -  selective   poisons    in, 

150 
symptomatology     of, 

general,  151 

treatment   of,    160 

•  optic,  82 

— ■ in    cerebral    abscesF, 

348 
•  postdiphtheritic,       course 

and    prognosis    of, 

158 

pathology   of,   158 

symptoms  of,  157 

retrobular,  83 

Neuro-fibromata,  162 

■ ■  intracranial,  352 

Neuromata,  162 

— —  ganglionic,  162 

plexiform,  162 

prognosis  of,  163 

symptoms  of,  163 

treatment  of,    164 

Neurone  of  Waldeyer,  1 

theory,  3 

Neurones,  association,  5 
classification   of,   5 


INDEX 


559 


Neurones,  fommissural,  5 
—  motor,  lower,  6 

effects  of  lesions 

of,  9 

- — upper,  5 

effects  of  lesiont 

of,  9 

projection,  5 

— — -  sensory,  11 
from   medullary    nu- 
clei  to   optic   thal- 
amus, 22 

•  from  optic  thalamus 

to   cortex.   23 

in  brain,  22 

in  peripheral  nerves, 

11 

in  posterior  roots,  14 

in  spinal  cord,  15 

Neuroses,  anxiety,  479,  504 

compulsion,  498 

occupation,  475 

vaso-motor,   531 

Ninth  nerve,  109 
Nodding  spasms,  470 
Nystagmus,  92 

in  aural  vertigo.  528 

in  cerebellar  tumotirs,  371 

in  disseminated  sclerosis, 

214 

in     Friedreich's     disease, 

240 

in  syringomyelia,  248 

in     tumours     of     corpora 

qnadrigemina,  366 

lesions  producing,   64 

miners',  94 

O 

Obsessions,  498 
Obstetrical  paralysis,  118 
Obturator  nerve,  141 

paralysis   of,   141 

Occipital   lobes,    functions    of, 
60 

tumours  of,  364 

■  pole,  lesion  at,  effect  of,  86 

Occupation  neuroses.   475 
Ocular  movements,  area  for,  59 

muscles,      paralysis      of, 

general  effects   of,   89 

nerves,  87 

— —  palsies  in  tabes,  302 

■ •  symptoms    in    amaurotic 

family  idiocy,  337 
i  n       cerebro  -  spinal 

fever,  321 
in  disseminated  scle- 
rosis, 214 
in      exophthalmic 

goitre,  538 
in   hysteria,   489 
in    migraine,    524 


Ocular  symptoms  in  myasthe- 
nia gravis,  422 
in  sinus  thrombosis. 

409 
in      tabes      dorsalis, 
300 
Oculo-cardiac  reflex.   50 
Oculo-motor  nerve,  87 
(Edema,   angioneurotic.  532 
(Esophageal  spasm,  490 
Olfactory  centre,  79 

nerve,  anatomy  of,  79 

■  stimulation    of.    79 

Ophthalmoplegia,  acute  infec- 
tive (see  Encephalitis  leth- 
argic a  J 
Ophthalmoplegic  migraine,  526 
Oppenheim's  reflex,  30 
Optic    atrophy,    83 

and   syphilis,    275 

hereditary,    84 

in  amaurotic  family 
idiocy.  337 

in  disseminated  scle- 
rosis,   214 

■  in      tabes      dorsalis, 

301 

primary,  83 

chiasma,   81 

and    cortex,     lesions 

between,    85 

lesions  of,  84 

disc,  ophthalmoscopic  ap- 
pearances 
of,  in  neii- 
ritis,  83 

in  optic  at- 
rophy, 84 

nerve,   anatomy  of,  80 

neuritis,  82 

•  from  general  toxae- 
mias, 83 

■  from     intracranial 

disease,  82 

in  cerebellar  tum- 
ours. 370 

in    cerebral    abscess, 

348 

tumours.    353 

i  n       cerebro  -  spinal 

fever,  321 

■  in      posterior     basic 

meningitis,  324 

•  in  tuberculous  men- 
ingitis, 314 

ophthalmoscopic  ap- 
pearances  in,   82 

radiation,  82 

thalamus,  connections  of, 
60 
functions  of.  61 
lesions  of.  61 

tumours  of.  366 

tracts,  81 


560 


INDEX 


Osteo-arthropatbies     in    tabes 

dorsalis,  295 
Osteoma,  intracranial    352 

P 

Pacliymeningitis.  cerehial,  312 

externa,  327 

hypertropbica,  261 

of  cord.  261 

Pains,  referred,  21 
Palate  reflex.  28 

Palsies  of  children,  cerebpllar, 
335 

cerebral,  329 

Paralyses,  bulbar,   186 

in  hysteria,  485 

of  brachial  plexns,   117 

of  lower  limbs,  140 

.         of  sinerle  roots,  117 

of  upper  limb,  115 

(,9cc  also  Palsies) 

Paralysis  agitans,   452 

etiology  of,  452 

_ pathology  of,  452 

prognosis  of,  458 

. symptoms  of.   453 

treatment  of,  458 
asthenic  bulbar  {see    My- 
asthenia gravis) 

Brown-Sequard,  21 

crossed  {see  Crossed  para- 
lysis) 

diver's,  206 

family   periodic,   473 

in  rheumatic  chorea,  462 

infantile    {see    Poliomye- 

litis, acute) 

ischaemic,   136 

Klumpke'6,  118 

Landry's,  198 

local,      following      super- 

ficial nerve  injuries,  148 
lower  plexus,   118 

of  anterior  crural  nerve, 

142 

of  calf  muscles.  145 

of  cranial   nerves  in   dis- 

seminated 
sclerosie,  214 
in      tabes     dor- 
salis,  302 

of  deltoid.  122 

of  external  rectus.  90 

of      facial      nerve      (.sec 

Facial   paralysis) 

of  inferior  oblique,  88 

rectus.   88 

of  internal  rectus.  90 

of  interossei,  131 

of  laryngeal  muscles,  110 

of  lips,  114 

of  long   subscapular 

nerve,  127 


Paralysis  of  musculo-spiral 
nerve,  129 

of  obturator  nerve,  141 

of  ocular  muscles,  89 

•  in  tabes.  302 

of  serratus  magnus.  124 

of  spinal  accessory  nerve. 

113 
■ of  sterno-mastoid,  113 

of    superior   oblique,    88 

rectus,  87 

of  third  nerve,  87 

in  migraine,  526 

of  tongue,  114 

of  trapezius,   113 

postepileptic,  430 

progressive    bulbar,     221. 

225.  226 
pseudo-bulbar,  186 

psoiido-hypertrophic,   166 

Paramyoclonus,  471 
Paraplegia,  183 

■  ataxic,  237 

— —  from  cortical  lesions,  185 

from  hysteria,  185 

from  spinal  cord  lesions, 

183 
during  war, 

40 

from    subcortical   lesions. 

184 

in  extension,  40 

— —  in  flexion,  40 

-  senile,  185 
■  spastic.  228 

hereditary,  243 

Parasympathetic  system,  ana- 
tomy of,  46 

functions  of.  48 

■ treatment  of  affec- 
tions  of,   53 

Parasyphilis  (see  Syphilis, 
parenchymatous;  General 
paralysis  of  insane ;  Tabes 
dorsalis) 

Parenchymatous  syphilis  (sec 
Syphilis,  parenchymatous) 

Parietal  lobe,  tumours  of.  363 

Parkinson's  disease  (see  Par- 
alysis agitans) 

mask,  454 

Patellar  clonus,  38 

jerk,  32 

Pawlow's  investigation  of 
higher  functions  of  ner- 
vous system,  62 

Pectoralis  major,  action  and 
atrophy  of,  124 

Perforating  ulcer  in  tabes,  293 

Periodic  paralysis,  family,  473 

Peripheral  facial  paralysis 
(see  Facial  paralysis, 
peripheral) 

nerves,  lesions  of,  120 


INDEX 


561 


Peripheral  nerves,  lesions  of, 
principles  of 
treatment  of, 
120 

symptoms  of,  121 

neuritis       (see      Neuritis, 

multiple) 
Peroneal    muscular    atrophy, 

176 
Petit  mal,  440 
Pharyngeal  reflex,   28,  35 
Phobias,  498  . 

Pigmentation      in      arsenical 

neuritis,  154 
Pithiatism,  483 

Pituitary   body,    anatomy   of, 
375 

diseases  of.  375 

secretions  of,  376 

tumours  of.  366 

Plantar  nerves,  145 

reflex,  27,  29 

crossed,  31 

in  children,  29 

. in  hysteria,  486 

value    of,     in     diag- 
nosis, 30 
Plexiform  neuromata,  162 
Polioencephalitis,   341 
Poliomyelitis,  acute,  189 

clinical  varieties  of, 

192 

diagnosis  of,   193 

etiology   of,   189 

pathology  of,   190 

prognosis   of,    194 

symptoms  of,  191 

treatment  of,   196 

Polyneuritis,      acute     febrile, 
159 

(see   Neuritis,  multiple) 

Pons    and    cerebellum,    angle 

of,  tumours  at,  367 

haemorrhage  into,  400 

tumours  of,  361 

Popliteal  nerve,  external,  143 

■ •  internal,   145 

Postdiphtheritic  neuritis,  157 

paralysis,  157 

Postepileptic  automatism,  438 
mania,  438 

paralysis,  438 

Posterior  basic  meningitis  (see 

Meningitis,        posterior 
basic) 
interosseous   nerve,    129 

thoracic  nerve,  lesions    of, 

124 

tibial  nerve,   145 

Post-hemiplegic  chorea,  399 
Pott's   disease,   233 
Precentral       convolution       of 

cerebrum,  58 
Preconscious  mind,  480 


Prefrontal  lobes,  functions  of, 

58 
Pressure  palsies,  129 
Primary    optic    atrophy,    and 

syphilis,  275 
Progressive   bulbar   paralysis, 
221,   225,  226 

lenticular      degeneration, 

386 
— —  muscular  atrophy,  219 

and  syphilis,  219 

diagnosis  of  bul- 
bar forms 
of,  225 

—  of  ocular 

forms   of, 
226 

o  f       spinal 

forms   of, 
224 

etiology   of,    219 

in   infants,    227 

prognosis  of,  226 

symptoms  of,  219 

treatment  of,  226 

Projection  fibres,    descending, 
5 

spheres,  54 

Pronator  jerk,  31 
Propriospinal     fibres     of     pos- 
terior columns,  18 

Protopathic  system,  12 
Psammoma  of  brain,  352 

of  spinal  cord,  254 

Pseudo  -  hypertrophic    paraly- 
sis, 166 

Psychasthenia     (see     Compul- 
sion  neuroses) 
Psychoanalysis,   480,   507 
Psycho-galvanic  reflex,  70 
Psychoneuroses,  479 

causation  of,  479 

internal    secretions    and, 

481 

treatment  of,   505 

bodily,   509 

by  hypnotism,   506 
by  persuasion,  506 

by      psychoanalysis, 

507 

by  suggestion,   506 

(see     also     Anxiety     neu- 
roses.  Compulsion    neu- 
roses, Hysteria,  Neuras- 
thenia) 
Ptosis,  congenital,  88 

in  hysteria,  489 

in     lesions     of      cervical 

sympathetic,  52 

in      myasthenia      gravis, 

422 

in  tabes  dorsalis,  302 

Pupil    in    lesions    of    cervical 

sympathetic,   52 


562 


INDEX 


R 

Rabies,  545 
Radial  nerve,  129 
Raynaud's  disease,  531 
Reaction  of  degeneration,  43 

times,  visual,  66 

—  in    p  a  V  a  1  y  s  is 

agitans,    67 
Recklinghausen's      (von)      dis- 
ease, 162 
Rectal    crises    in    tabes    dor- 

salis.  300 
Referred  pains,  21 

in    acute    abdominal 

disease,   21 
Reflex,  bladder,  36 

conditional,  62 

crossed  plantar,  31 

laryngeal,  35 

mass,   36 

nervous  disorders,   148 

pharyngeal,  35 

plantar,    29 

in  children.  29 

psycho-galvanic,  70 

rectal,  36 

Reflexes,  deep,  26,  31 

mechanism  of,  25 

■  superficial,   27 

—  abdominal,  in  tum- 
ours of  frontal 
lobes,  363 

primitive  forms  of,  25 

tendon,  26,  31 

visceral,  35 

Reminiscences  in  epilepsy,  437 

Renal  crises,  300 

Respiratory      arrhythmia      of 

pulse,  50,  502 

Rest  treatment  in  exophthal- 
mic goitre,  541 

i  n      psychoneuroses, 

511 

Retrobulbar  neuritis,  83 

Retropulsion,   457 

Rheumatic  chorea,  460 

Rigidity  and  contractures,  39 

decerebrate,   40 

in  hemiplpgia,   398 

in   hysteria,   489 

in   paralysis   agitans;   454 

prevention      and      treat- 
ment of,  41 

theories  of   cause  of,   39 

Romberg'e  sign,  292 
Rubro-spinal  tract,  7 
lesions  of,  10 


Sach's  disease,  336 

Sacral  plexus,  distribution  of, 

140 
St.  Vitus's  dance  (f^cc  Chorea) 


Saltatory  chorea,  491 
Salvarsan  in   general   paraly- 
sis of  the  insane,  282 

in  tabes  dorsalis,  307 

Saicoma,  intracranial,  352 

of  spinal  cord,  254 

Scapular  reflex,  28 

Sciatic  nerve,  great,  course 
and  paralysis  of, 
143 

small,  142 

Sciatica,   146 

diagnosis  of,  147 

etiology  of,  146 

pathology  of,  146 

prognosis  of,  148 

symptoms  of,  146 

treatment  of,  147 

Scissors-legged  progression,  39, 

331 
Sclerosis,  amyotrophic  lateral, 

234 
combined,    and    syphilis, 

275 

disseminated,    210 

lateral,  228 

and  syphilis,  228,  234, 

275 
Secondary  deviation,   90 
Senile  chorea,   466 

paraplegia,  185 

Sensibility,    deep,  system  for, 

12 

epicritic,  12 

loss  of,  13 

protopathic,  12 

loss  of,  13 

Sensitization     of     tissues      in 

syphilis,  276 
Sensory   changes    in   tumours 
of  motor  cortex,  358 

impulses,  area  for,  60 

neurones    {see    Neurones, 

sensory) 
Serous  meningitis,  325 
Serratus  magnus,  paralysis  of, 

124 
Serum,  immunized,  in  general 
paralysis      of      in- 
sane, 283 

in  tabes  dorsalis,  307 

Seventh  nerve,  98 
Single-system  lesions  of  cord, 

180 
Sinus  thrombosis,   408 

cavernous,    408 

lateral.    408 

longitudinal,   410 

Sixtii    nerve,   anatomy  of.   89 

paralysis  of,  89 

Skew   deviation  of  eyes,  371 
Smell  and  taste,  area  for,  60 

subjective   sensations    of, 

in  cerebral  tumours,  366 


INDEX 


563 


Smell,     subjective    sensations 

of,  in  epilepsy,  436 
Spasm,  hysterical,  489 

of  facial  muscles,  106 

of  laryngeal  muscles,  112 

Spasmus  nutans,  470 
Spastic  paraplegia,  228 

diagnosis  of,  230 

etiology  of,  228 

hereditary,  243 

pathology  of,  233 
primary,   228 
prognosis  of,  233 
secondary,   231 
symptoms  of,  228 
syphilis  and,  228 
treatment  of,   233 
Speech,  area  for,  58 

auditory  centre  for,  60 

Broca's  convolution  and, 

418 

"  centres  "  for,  416 

in  disseminated,  sclerosis, 

214 
in     Friedreich's     disease, 

240 
in    general    paralysis    of 

insane,  280 

motor  centre  for,  413 

tics  of,  468 

Spina  bifida,  251 

•  occulta,   251 

treatment  of,  252 

varieties  of,  251 

Spinal   accessory   nerve,   ana- 
tomy of,  112 
— paralysis  of,  113 

caries,  233 

cord  anaesthesia,  types  of, 

20 

classification  of  dis- 
eases of,  179 

haemorrhage  into,  252 

irregular  lesions  of, 

179.  182 

non  -  system     lesions 

of,  179,  182 

path  of  impulses  in, 

19 

segmentary    sensory 

areas  of,  15 

sensory  neurones  in, 

15 

syphilis,  271,  274  (see 

also  General  para- 
lysis of  insane; 
Tabes  dorsalis) 

system     lesions     of, 

180 

tracts  of,  15 

tumours  of,  254 

meningitis,  261 

diagnosis  of,   262 

forms  of,  261 


Spinal    meningitis,    prognosis 
of,  263 

symptoms  of,   261 

treatment  of,   263 

Spine,  caries  of,  233 
Spirochaeta   pallida,   in   brain 
and     spinal     cord, 
267 

persistence      of,      as 

"  rests,"  276 
Spironema  pallidium  (see  Spi- 
rochaeta pallida) 
"  Spontaneous  pains  "  in  tha- 
lamic lesions,  61 
Spotted    fever     (see     Cerebro- 
spinal fever) 
Squint,   89 

Status  epilepticus,  439 
Stellwag's  sign,  539 
Stereognosis,  14,  288,  364 
Sterno-mastoid  paralysis,  113 
Strabismus,  89 

Structure  of  nervous  system,  1 
Subacute  combined  degenera- 
tion. 207 
diagnosis  of,  208 
pathology  of,  208 
prognosis  of,  209 
symptoms  of  ,207 
treatment  of,  209 
Subconscious  mind,  480 
Suggestion,   506 
Superficial  anal  reflex,  27 

reflexes,  27 

Superior  gluteal  nerve,  142 

obliciue,  paralysis  of,   88 

rectus,  paralysis  of,  87 

Supinator  jerk,  31 
Suppurative  encephalitis,  346 

meningitis,  326 

Sydenham's  chorea  (see  Cho- 
rea) 

Sympathetic  system,  cervical, 
51 

paralysis   of,   52 

vertebral,  46 

^ anatomy  of,  49 

endocrine  secre- 
tions and,  53 

symptoms  of  dis- 
turbance of,  49 

treatment  of  af- 
fections of,  53 

Sympatheticotonus,  49 

Synergia,  dependence  of,  on 
cerebellum,   64 

Syphilis,  and  cerebral  haemor- 
rhage, 389 

and    combined    sclerosis. 

275 
and  lateral  sclerosis.  228, 

234,   275 
and  periodic  epileptiform 

attacks,   275 


561 


INDEX 


Syphilis,    and    primary    optic 
atrophy,   275 

and  progressive  muscular 

atrophy,  219,  275 

interstitial,  of  brain,  269, 

of  cord,  271 

diagnosis  of,  273 

gummata  in,  273 

lesions  in,  271 

prognosis  of,  274 

meningo-vascularis,  269 

of  nervous  system,  266 

cerebro  -  spinal 

fluid  in,  267 
forms  of.  266 
ealvarsan  in,  269 
sensitization    of 

tissues  in,  276 
"Was  serma  n  n 

test  in,  268 

parenchymatous.   266,  274 

—  course  of,  275 

distribution  of  spiro- 

chaetes  in,  by  peri- 
neural lymphatics, 
277 

latency       of       spiro- 

chsetes  in,  276 

stages  of,  277 

Wassermann  test  in. 

275 

Syphilitic  myelitis,  acute,  273 

Syringomyelia,  246 

diagnosis   of,   250 

etiology  of,   246 

pathology  of,  246 

prognosis  of,  250 

symntoms  of,  247 

treatment  of,  250 

Syringomyelocele,  251 


Tabes  dorsalis,  atrophy  of 
bones  in,  299 

Charcot's    joints    in, 

295 

clinical  course  of,  290 

— —  crises  in,  299 

diagnosis  in,  302 

epileptiform    fits    in, 

300 

—  etiology  of,  284 

hypotonus  in,  288 

mechanism  of  pro- 
duction of  symp- 
toms in.  287 

ocular  symptoms  in, 

300 

—    ostoo  -  arthropathies 

in.  295 
pains  in,  288,  290 


Tabes  dorsalis,  pathology  of, 

286 
perforating  ulcer  in, 

293 
preataxic    stage    in, 

291 

prognosis  of,  305 

selective     action     of 

toxin  in,  285 

symptoms   of,   287 

treatment  of,  306 

trophic    changes    in, 

293 

juvenile,   284 

Tabo-paresis,  274 
Tachycardia  in  exophthalmic 

goitre,  538 
Taste,    connections    of    nerve 

for,  96 
Tecto-spinal  tract,  7 

lesions  of,  10 

Teichopsia,  524 
Telescopic  vision,  86 
Temperature  in  cerebral  haem- 
orrhage, 395 

in  hysteria,  497 

Temporal  lobe,  functions  of,  60 
Temporo-sphenoidal  lobes,  tum- 
ours of,  365 
Tendo-Achillis  jerk,  32 

• •  in  sciatica,  147 

Tendon  reflexes,  26,  31 
Tenth  nerve,  109 
Tetanus.    542 

diagnosis  of,  543 

etiology  of,  542 

pathology  of,  542 

prognosis  of,  544 

symptoms  of,  542 

treatment    of,    544 

■ antitoxic.   544 

Tetany,  449 

diagnosis  of,  450 

etiology  of,  449 

pathology  of,  449 

prognosis  of,  450 

symptoms  of,  449 

treatment  of.  451 

Thalamo-spinal  tract,  7 

lesions  of,  10 

Third  nerve,  anatomy  of,  87 

paralysis  of,  87 

Thirst,  abnormal,  in  cerebral 

tumour,  366 
Thomson's  disease,  472 
Thoracic  nerve,  long.  124 
Thrombosis,  cerebral.  401 
— —  of  cerebral  sinuses,  408 
Thymus  gland  in  myasthenia 

gravis,  421 
Thyroid    extract    in    acrome- 
galy, 384 

gland     in     exophthalmic 

goitre,  537 


INDEX 


565 


Tibial  nerve,   anterior,   144 

posterior,  145 

Tic  douloureux,  516 
Tics,  467 

characters   of,   468 

coordinate,  467 

diagnosis  of,  469 

etiology  of,   468 

mental,  467 

of  speech,  468 

prognosis  of,  469 

simple,  467 

treatment  of.  470 

Time  discrimination,  67 
Tongue,  deviation  of,  in  hemi- 
plegia,   397 

in      myasthenia      gravis, 

424 
Torticollis,  spasmodic,  diagno- 
sis of,  from  tics,  469 
Tract,  direct  cerebellar,  18 

of  Gowers,  19 

rubro-spinal,  7 

lesions  of,  10 

spino-thalamic,  19 

tecto-spinal,  7 

lesions  of,  10 

thalamo-spinal,   7 

lesions  of.  10 

ventral  cerebellar,  19 

vestibulo-spinal,  7 

lesions  of.    10 

Tracts,   ascending  antero-late- 

ral,  19 

motor,     clinical     import- 

ance of,  8 

pyramidal,  6 

subsidiary        descending, 

symptoms  of  disease  of, 

10 
Transverse  myelitis,  201 
Trapezius,  paralysis  of,  113 
Traumatic  neurasthenia,   502 
Tremors,   fibrillary,   221 
— — -  in  cerebellar  disease,  371 

in    exophthalmic    goitre, 

539 

in    general    paralysis    of 

insane,  280 

in  hysteria,  488 

in    mercurial    poisoning, 

217 

in  paralysis  agitans,  453 

in  tumours  of  crus  cere- 

bri, 361 

of  frontal  lobes,  363 

Triceps  jerk,  32 

Trigeminal     nerve,     anatomy 

of,  94 
lesions  of,  95 

neuralgia,  516 

Trismus,   542 

Trochlear  nerve,  anatomy  and 
lesions  of,  88 


Trophic   symptoms   in   arseni- 
cal neuritis,  154 

in   multiple  neuritis, 

152 

■ in  myelitis,  202 

in  syringomyelia,  247 

in  tabes  dorsalis,  293 

Tuberculoma   of    spinal   cord, 

254 
Tuberculous     deposits,     intra- 
cranial, 351 
Tumours,  cerebellar,  effects  of, 

direct,  370 
indirect,  372 

cerebral     (see     Tumours, 

intracranial) 

extramedtillary,  254 

intracranial,  351 

diagnosis  of,  372 

localization  of,  355 

at  angle  of  pons 

and      cerebel- 
lum, 367 

at  base  of  brain, 
366 

in  cerebellum, 
370 

in  corpora 
Quadrigemina, 
366 

in  corpus  callo- 
sum,  366 

in  cortical  mo- 
tor area,   356 

in  crus  cerebri, 
360 

in  frontal  lobes, 
363 

in  medulla,  362 

in  occipital 
lobes,  364 

in  optic  thala- 
mus, 366 

in  parietal 
region,  363 

in  pituitary 
body,  366 

in  pons,  361 

in  region  of  in- 
ternal     cap 
eule,   360 

in  t  e  m  p  o  r  o- 
sp  he  noi  da 
lobes.  365 

in  uncinate  gy 
rus,  365 
prognosis  of,  373 
symptoms  of,  genes 
al.  352 

local,  355 

treatment  of.  373 
varieties  of,  351 
of  Cauda  equina,  257 
of  nerves,  162 


566 


INDEX 


Tumours  of  spinal  cord,  254 

diagnosis  of,  257 

localization     of, 

257 

symptoms  of, 255 

treatment  of,  259 

varieties  of,  254 

intramedullary,  254 

Tuning-fork  tests,   528 
Twelfth  nerve,  113 


U 


Ulcers,   perforating.   293 
Ulnar  nerve,  course  of,  130 

paralysis  of,  131 

Uncinate  convolution,  60 

gyrus,  functions  of,  60 

tumours  of,  365 

Unconscious  mind,  479,  480 
Uniradicular  palsies,  117 
Upper  limb,  paralyses  of,  115 


Vagus,  anatomy  of,  109 

lesions  of,  110 

Vaso-motor  neuroses,  531 

in  hysteria,  496 

in  multiple  neuritis, 

152 
of    general   distribu- 
tion,   535 
Vegetative   system   (see  Auto- 
nomic system) 
Vertebral  arteries,  387,  388 

sympathetic    system    (sec 

Sympathetic      system, 
vertebral) 
Vertigo,  527 

aural,  527 

diagnosis  of,  529 

— — •  prognosis  of,  529 

symptoms   of,    528 

treatment  of,  529 

etiology  of,  527 

lesions  producing,   64 

Vestibular  nerve,  109 

nucleus,    connections    of, 

with   cerebellar   nuclei, 

63 
Vestibulo-spinal    tract,    7 

• —  lesions  of,  10 

Vibration,  sense  of,  14 
Visceral  reflexes,  27,  35 
Visual  centres,  60.  412,  414 
—  lesions  of,  86 

paths,  80 

symptoms      in      cerebral 

tumours.    353 

in  epilepsy,  436 

i  n      exopbthalm  i  c 

goitre,  538 


Visual  symptoms  in  hysteria, 
493 

in  migraine,   524 

Vocal  cords,  paralysis  of,   110 
Volkmann's   contracture,    136 

•  diagnosis  of,   138 

etiology  of,   136 

pathology  of,   137 

prognosis  of,  139 

symptoms  of,  136 

treatment  of,   139 

Vomiting    in    postdiphtheritic 

neuritis,  157 
Von  Graefe's  sign,  539 
Von   Eecklinghausen's  disease, 

162 

W 

War   injuries  to   spinal  cord, 

35,  40 
Waren  Tay's  disease,  336 
Wassermann    reaction    in    in- 
terstitial   syphilis, 
268,  269 

in     parenchymatous 

syphilis,     268,     275, 
278 

modification    of,    by 

treat- 
ment,  in 
intersti- 
t i  a  1  sy- 
philis, 268 

in    paren- 

cliymat- 
ous  syphi- 
lis,  268 
Wernicke's  aphasia.  418,  419 

hemiopic   pupil   reaction, 

86 

zone,  417 

"  Wet   brain,"   325 
Word-association  tests,  Jung's, 

70,  508 
Word-blindness,    414 
Word-deafness,  414,  416 
Wrist-drop,  156 
Writer's  cramp,  475 

diagnosis  of,  477 

prognosis  of,  478 

seat  of  lesions  in,  476 

symptoms  of,  476 

treatment  of.  478 

•  writing  in,  476 

Writing  centre,  in  aphasia,  414 

in    general     paralysis    of 

insane,  280 

in  paralysis  a^itane,  453 

in  writer's  cramp,  476 


Z 


Zone  of  Wernicke,  417,  418,  419 


Printed  by 

Cassell  &  Company,  Limiied,  La  Belle  Sauvage. 

London,  E.C.4 

30.521 


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rangement with  the  Librarian  in  charge. 

DATE  BORROWED 

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DATE  DUE 

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MAY  1     '^' 

m 

tri^f  -^ 

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C28II  140)MI00 

TS6 
1921 


